AMENDED AND RESTATED BIOMARIN PHARMACEUTICAL INC. NONQUALIFIED DEFERRED COMPENSATION PLAN

1.

DEFINITIONS.

1.1

"Account" means the book entry account(s) established under the Plan for each Participant’s Compensation Deferrals, Discretionary Employer Contributions and any contribution credits and deemed income, gains and losses credited thereto or debited therefrom. Account balances shall be reduced by any distributions made to the Participant or the Participant’s Beneficiary(ies) therefrom and any charges that may be imposed on such Account(s) pursuant to the terms of the Plan. Separate Subaccounts may be established to which shall be credited a Participant’s Compensation Deferrals for each separate Plan Year, the Discretionary Employer Contributions, if any, and the gains and losses with respect thereto. Where Subaccounts have been established, Account shall refer to all of the Participants’ Subaccounts, collectively, as the context may require.

1.2

"Affiliate" means, with respect to any entity, all other entities with which the subject entity would be aggregated and treated as a single employer under Code Section 414(b) (controlled group of corporations) and Code Section 414(c) (a group of trades or businesses, whether or not incorporated, under common control), as applicable.

1.3

"Beneficiary" means any person or persons so designated in accordance with the provisions of Section 7.1.

1.4

"Board" means the Board of Directors of the Company. If one or more committees have been appointed by the Board to determine eligibility under the Plan, Discretionary Employer Contributions to be made to the Plan, or to exercise any other Company discretion with respect to such Plan, "Board" also means such committee(s).

1.5

"Change Of Control" shall mean either (i) a merger, consolidation, share exchange, business combination, issuance of securities, direct or indirect acquisition of securities, tender offer, exchange offer or other similar transaction as a result of which the persons that beneficially owned, directly or indirectly, the shares of the Company’s voting stock immediately prior to such transaction cease to beneficially own, directly or indirectly, shares of voting stock representing more than fifty percent (50%) of the total voting power of all outstanding classes of voting stock of the Company or the continuing or surviving corporation if Company is not the continuing or surviving corporation in such transaction, or (ii) a sale of all or substantially all of the assets of Company.

1.6

"Code" means the Internal Revenue Code of 1986 and the regulations thereunder, as amended from time to time.

1.7

"Committee" means the Administrative Committee composed of such individuals as may be appointed by the Board which shall function as the administrator of the Plan.

1.8

"Company" means BioMarin Pharmaceutical Inc., a Delaware company, and any successor organization thereto.

1.9

"Compensation" means, in the case of a Participant who is an employee of the Company such Participant’s regular cash salary and cash bonuses, and Restricted Stock Compensation, and in the case of a Participant who is a Director of the Company, such Participant’s annual Board retainer and Board meeting fees (including Board Committee meeting fees). Compensation shall also include any "Performance Based Compensation" as that term is defined under Section 409A of the Code and any regulations thereunder.

1.10

"Compensation Deferrals" means the percentage or dollar amount of an Eligible Employee’s Compensation which the Eligible Employee elects to defer pursuant to Section 3.1 .

1.11

"Designation Date" means the date or dates as of which a designation of deemed investment directions by an individual pursuant to Section 4.3, or any change in a prior designation of deemed investment directions by an individual pursuant to Section 4.3 , shall become effective. The Designation Dates in any Plan Year shall be determined by the Committee.

1.12

"Director" means a non-employee member of the Board of Directors.

1.13

" Disability " will be determined to exist if the Participant is, by reason of any medically determinable physical or mental impairment which can be expected to result in death or can be expected to last for a continuous period of not less than twelve (12) months, eligible to receive income replacement benefits for a period of not less than three (3) months under any disability benefit plan for covered Employees of the Employer, or, if the Participant does not participate in such plan, would have been eligible to receive such benefits had the Participant participated in such plan.

1.14

"Discretionary Employer Contributions" means the amount, if any, of contributions awarded to a Participant pursuant to Section 3.2 .

1.15

"Effective Date" means the effective date of the Plan, which shall be December 1, 2005, or if later, the date the Plan is approved by the Board.

1.16

"Election" means the form on which a Participant (i) elects to make Compensation Deferrals pursuant to Article 3 or (ii) elects a fixed payment date pursuant to Article 5 , or (iii) elects the method by which his or her Account will be distributed pursuant to Article 6. The Election shall be in such form, including specifically by electronic means, as may be prescribed by the Committee.

1.17

"Eligible Employee" means, for any Plan Year (or applicable portion thereof), an employee of the Employer who is a member of the select group of management or highly compensated employees as more particularly described in Article 2 and who has been designated by the Committee, in its sole discretion, as eligible to participate in the Plan.

1.18

"Employer" shall be defined as follows:

(a)

Except as otherwise provided in part (b) of this Section, the term "Employer" shall mean the Company and/or any of its subsidiaries or affiliates (now in existence or hereafter formed or acquired) that have been selected by the Committee to participate in the Plan and have adopted the Plan for the benefit of its Eligible Employees.

(b)

For the purpose of determining whether a Participant has experienced a Separation from Service, the term "Employer" shall mean the entity for which the Participant performs services and with respect to which the legally binding right to compensation deferred or contributed under this Plan arises, and all of its Affiliates.

1.19

"Entry Date" means the first day of any Plan Year and, as to any Eligible Employee, the date which is thirty (30) days from the date on which such Eligible Employee is first notified by the Committee of his or her eligibility to participate in the Plan. Notwithstanding the foregoing, for any individual first designated as an Eligible Employee on or before the Effective Date, his or her Entry Date shall be the Effective Date

1.20

"Open Enrollment Period" means such period as the Committee may specify which ends prior to the first day of each Plan Year, or, with respect to an Eligible Employee or Director who first becomes eligible to participate in the Plan during a Plan Year, ends within thirty (30) days of becoming an Eligible Employee or Director, provided that the newly Eligible Employee or Director was not previously eligible in another individual account deferred compensation plan that would be aggregated with this Plan pursuant to Treasury Regulation Section 1.409A-1(c) at any time during the 24-month period ending on the date he or she became eligible to participate in the Plan. If an Eligible Employee or Director first becomes eligible after the first day of the Plan Year, the Compensation Deferral shall be limited to Compensation paid for services performed after the date of the Election. For Compensation that is earned based upon a specific performance period (e.g., annual bonus or retainer), a Compensation Deferral shall be limited to Compensation paid for services performed after the Election if the Compensation Deferral applies to no more than an amount equal to the total amount of the Compensation for the performance period multiplied by the ratio of the number days remaining in the performance period after the Election over the total number of days in the performance period. Notwithstanding the foregoing, the Open Enrollment Period for deferrals of Performance Based Compensation may end no later than six (6) months prior to the end of the performance period for which services are to be rendered.

1.21

"Participant" means an Eligible Employee or Director who has elected to participate in the Plan by executing and submitting an Election to the Committee. A Participant shall also mean an Eligible Employee for whom Discretionary Employer Contributions are made, regardless of whether such Eligible Employee has executed and submitted an Election.

1.22

"Performance-Based Compensation" means any incentive bonus or other compensation amount to the extent that it is (a) variable and contingent on the satisfaction of pre-established organizational or individual performance criteria, (b) not readily ascertainable at the time the deferral election is made, and (c) based on services performed over a period of at least twelve (12) months. For this purpose, performance criteria are "pre-established" if they are established in writing no later than ninety (90) days after the related service period begins.

1.23

"Plan" means this BioMarin Pharmaceutical Inc. Nonqualified Deferred Compensation Plan, as amended from time to time.

1.24

"Plan Year" means the twelve (12) month period beginning on each January 1 and ending on the following December 31.

1.25

"Restricted Stock Compensation," means any restricted stock, restricted stock unit, phantom stock or similar award granted by the Employer to a Participant under any Employer-sponsored equity compensation plan.

1.26

"Retirement" means, in the case of a Participant employed by the Company, Separation from Service on or after age 55 with 10 years or more of service, and in the case of a Director, Separation of Service as a Director.

1.27

"Separation from Service" (or "Separates from Service" ) shall mean the termination of services provided by a Participant to his or her Employer, whether voluntarily or involuntarily, as determined by the Committee in accordance with Treasury Regulation Section 1.409A-1(h). In determining whether a Participant has experienced a Separation from Service, the following provisions shall apply:

(a)

For a Participant who provides services to the Employer as an Employee, except as otherwise provided in Section 1.27(b) , a Separation from Service shall occur when such Participant experiences a termination of employment with such Employer. A Participant shall be considered to have experienced a termination of employment when the facts and circumstances indicate that either (i) the Participant is not reasonably expected to perform further services for the Employer after a certain date, or (ii) that the level of bona fide services the Participant will perform for the Employer after such date (whether as an Employee or as an independent contractor) will permanently decrease to no more than 49% of the average level of bona fide services performed by such Participant (whether as an Employee or an independent contractor) over the immediately preceding 36-month period (or full period of services to the Employer if the Participant has been providing services to the Employer for less than 36 months).

(b)

If a Participant is on military leave, sick leave, or other bona fide leave of absence, the employment relationship between the Participant and the Employer shall be treated as continuing intact, provided that the period of such leave does not exceed six months, or longer, so long as the Participant retains a right to reemployment with the Employer under an applicable statute or by contract. If the period of leave exceeds six (6) months and the Participant does not retain a right to reemployment under an applicable statute or by contract, the Participant will incur a Separation from Service as of the first day immediately following the end of such 6-month period. However, where a Participant’s leave of absence is due to his or her inability to perform the duties of his or her position or any similar position as the result of any medically determinable physical or mental impairment that can be expected to result in death or last for a continuous period of not less than six (6) months, a 29-month period of absence will be substituted for such 6-month period. In applying the provisions of this paragraph, a leave of absence shall be considered a bona fide leave of absence only if there is a reasonable expectation that the Participant will return to perform services for the Employer.

(c)

Notwithstanding the foregoing, if a Participant who provides services to the Employer as both an Employee and a member of the Board, then to the extent permitted by Treasury Regulation Section 1.409A-1(h)(5), the services provided by such Participant as a Board member shall not be taken into account in determining whether the Participant experiences a Separation from Service as an Employee, and the services provided by such Participant as an Employee shall not be taken into account in determining whether the Participant has experienced a Separation from Service as a Board member.

(d)

Notwithstanding the foregoing, if, in connection with the Employer’s sale of substantial assets to an unrelated buyer (within the meaning of Treasury Regulation Section 1.409A-1(h)(4)), a Participant would otherwise experience a Separation of Service, then, in accordance with Treasury Regulation Section 1.409A-1(h)(4), the asset purchase agreement may specify whether or not such Participant has experienced a Separation from Service, provided that all Participants affected by the asset sale are treated consistently under the Plan.

1.28

"Trust" means any trust, including a grantor trust within the meaning of subpart E, part I, subchapter J, chapter I, subtitle A of the Code, created by the Trust agreement, to hold Compensation Deferrals and Discretionary Employer Contributions.

1.29

"Trustee" means the trustee of the Trust described in Article 11 .

1.30

"Valuation Date" means any business day on which the New York Stock Exchange is open, or such other date that the Committee, in its sole discretion, designates as a Valuation Date.

2.

ELIGIBILITY AND PARTICIPATION .

2.1

Eligibility. Eligibility for participation in the Plan shall be limited to Directors and a select group of management or highly compensated employees of the Employer, who are designated by the Committee, in its sole discretion, as eligible to participate in the Plan. Eligible Individuals shall be notified as to their eligibility to participate in the Plan. Participation in the Plan is voluntary, other than for Discretionary Employer Contributions. A person shall cease to be an Eligible Person for future plan years at such a time as he or she is neither a member of a select group of management nor highly compensated employees of the Company nor a Director of the Company.

2.2

Commencement Of Participation. An Eligible Employee may begin participation in the Plan upon any Entry Date, subject to the execution and submission of an Election pursuant to Article 3 . In addition, participation of an Eligible Employee who has not otherwise commenced participation in the Plan, shall commence when a Discretionary Employer Contribution is made to the Account of such Eligible Employee pursuant to the provisions of Section 3.2.

2.3

Cessation Of Participation. Active participation in the Plan shall end when a Participant’s employment terminates for any reason or at such time as a Participant is notified by the Committee pursuant to Section 2.4 that he or she is no longer eligible to participate in the Plan. Upon Separation from Service of employment or termination of eligibility, a Participant shall remain an inactive Participant in the Plan until the vested Account of the Participant under this Plan has been paid in full.

2.4

Change Of Employment Category. During any period in which a Participant remains with the company, but ceases to be an Eligible Person, he or she shall not be eligible to make Compensation Deferrals, or to receive Discretionary Employer Contributions hereunder for future plan years.

3.

CONTRIBUTIONS AND CREDITS .

3.1

Participant Contributions And Credits .

(a)

Time and Manner of Election. In accordance with rules established by the Committee, a Participant may elect to defer Compensation that would otherwise be paid to the Participant that is attributable to services first performed after the end of the applicable Open Enrollment Period. Notwithstanding the foregoing, the Open Enrollment Period for deferrals of Performance Based Compensation may end no later than six (6) months prior to the end of the performance period for which services are to be rendered. A Participant who is a Director may elect to defer up to 100% of his or her Compensation earned during the Plan Year. Amounts so deferred shall be considered a Participant’s "Compensation Deferrals" and shall be deducted by the Company from the Compensation of the deferring Participant and shall be credited to the Compensation Deferral Account of the deferring Participant. The Participant may, on an applicable election form provided by the Committee, elect for his or her Compensation deferrals to be paid in a lump sum within a specific calendar year, subject to such limitations as the Committee may set forth in the applicable election form. In addition, if permitted by the Committee, a Participant may elect to receive a distribution in annual installments. The annual installments shall commence within a specific calendar year as set forth within the election form. Furthermore, in accordance with rules established by the Committee, a Participant may elect to defer Restricted Stock Compensation that would otherwise be payable to the Participant to the Participant’s Account. The value of the Restricted Stock Compensation to be credited to the Participant’s Account shall be equal to fair market value of the number of shares vesting to the Participant as of the date of the shares vesting.

(b)

Timing of Election. The Election must be filed with the Committee during the Open Enrollment Period for the Plan Year to which such Election applies. Elections to defer Restricted Stock Compensation must be made no later than twelve (12) months prior to the vesting of the shares and no later than thirty (30) days after the granting of the award.

(c)

Irrevocable Election. The Participant’s Election with respect to his or her Compensation Deferrals is irrevocable. Unless increased, decreased or terminated during any subsequent Open Enrollment Period, an Election shall remain in effect until so changed by the Participant during such subsequent Open Enrollment Period.

(d)

Limitation on Compensation Deferrals. A Participant’s Compensation Deferral Elections shall be subject to the following:

(i)

A Participant electing to defer compensation for a given Plan Year must elect to defer a minimum of the greater of 1% of his or her Compensation or $10,000 each Plan Year.

(ii)

A Participant may elect to defer up to a maximum of one hundred percent (100%) of his or her Compensation and 100% of Performance Based Compensation.

(iii)

For each Plan Year in which a Compensation Deferral is being withheld from a Participant, the Participant’s Employer(s) shall withhold from that portion of the Participant’s Compensation that is not being deferred, in a manner determined by the Employer(s), the Participant’s share of FICA or other employment taxes on such Compensation Deferral. If necessary, the Committee may reduce the Compensation Deferral in order to comply with this Section.

(e)

No Withdrawal. Except as provided in Section 5.2 , amounts credited to a Participant’s Account may not be withdrawn by a Participant and shall be paid only in accordance with the provisions of this Plan and applicable Participant Election.

(f)

Vesting . A Participant shall at all times be 100% vested in the amounts credited to his or her Compensation Deferral Account.

3.2

Discretionary Employer Contributions And Credits .

(a)

Discretionary Employer Contributions. Apart from Compensation Deferral Contributions, the Board shall retain the right to make discretionary contributions for any Participant under this Plan at the times and in the amount(s) designated by the Employer, in its sole discretion. Amounts so credited will be considered a Participant’s "Discretionary Employer Contributions."

(b)

Company Contribution Account. There shall be established and maintained a separate Company Contribution Account in the name of each Participant to which shall be credited the amount of any Company Contributions during a plan Year and any earnings thereon, and from which shall be debited the amount of any losses thereon and the amount of any distributions made to the Participant therefrom.

(c)

Vesting. Amounts credited to the Company Contribution Account shall become 100% vested after the Participant has had three full Years of Service with the Company following the Plan Year in which the Company Contribution was made, or at such other time as

the Company may designate at the time the Contribution is made. Notwithstanding the preceding, if (i) the Participant dies or incurs a Disability prior to vesting or (ii) there is a Change of Control, all amounts credited to his or her Company Contribution Account shall become 100% vested. Any Participant that experiences a severance prior to full vesting shall irrevocably forfeit the portion not vested at the time of severance, and the amount so forfeited shall be returned to the Company. Any Participant that experiences a Retirement prior to full vesting shall, unless the Committee determines otherwise in its sole discretion (which shall include the discretion to fully vest amounts credited to the Company Contribution Account on Retirement), irrevocably forfeit the portion not vested at the time of Retirement, and the amount so forfeited shall be returned to the Company.

(d)

Forfeitures for Misconduct. If a Participant Separates from Service with the Employer as a result of the Participant’s gross misconduct, as determined by the Committee, or if the Participant engages in unlawful business competition with the Employer, the Participant shall forfeit all amounts allocated to his or her Discretionary Employer Contribution Account(s) under this Section 3.2 (regardless of the vesting of such amounts). Such forfeitures shall be retained by the Employer.

4.

ALLOCATION OF FUNDS .

4.1

Allocation Of Deemed Earnings Or Losses On Accounts. Subject to such limitations as may from time to time be required by law, imposed by the Committee or the Trustee or contained elsewhere in the Plan, and subject to such operating rules and procedures as may be imposed from time to time by the Committee, prior to the date on which a direction will become effective, the Participant shall have the right to direct the Committee as to how amounts in his or her Compensation Deferral Account shall be deemed to be invested. However, with regard to Restricted Stock Compensation the amount deferred will be deemed to be invested in Company stock and will remain in Company stock until such amount is distributed in shares to the Participant pursuant to Section 6. The Committee may, but is not required to, invest assets held by the Company on behalf of the Participant pursuant to the deemed investment directions the Committee has properly received from the Participant, and may utilize the Trust for the same in its discretion.

4.2

Accounting For Distributions. As of the date of any distribution hereunder, the distribution made hereunder to the Participant or his or her Beneficiary or Beneficiaries shall be charged to such Participant’s Account. Such amounts shall be charged on a pro rata basis against the investments of the Plan in which the Participant’s Account is deemed to be invested.

4.3

Deemed Investment Directions Of Participants. Subject to such limitations as may from time to time be required by law, imposed by the Employer or the Trustee or contained elsewhere in the Plan, and subject to such operating rules and procedures as may be imposed from time to time by the Employer, prior to and effective for each Designation Date, each Participant may communicate to the Employer a direction (in accordance with (a), below) as to how his or her Account should be deemed to be invested among such categories of deemed investments as may be made available by the Employer hereunder. Such direction shall designate the percentage (in any whole percent multiples) of each portion of the Participant’s Account which is requested to be deemed to be invested in such categories of deemed investments, and shall be subject to the following rules:

(a)

Any initial or subsequent deemed investment direction shall be in wr