BIOBLAST PHARMA LTD. NOTICE OF ANNUAL AND EXTRAORDINARY GENERAL MEETING OF THE SHAREHOLDERS Notice is hereby given that an Annual and Extraordinary General Meeting of the Shareholders of Bioblast Pharma Ltd. (" Company "

BIOBLAST PHARMA LTD.

NOTICE OF ANNUAL AND EXTRAORDINARY GENERAL

Notice is hereby given that an Annual and

Extraordinary General Meeting of the Shareholders of Bioblast Pharma Ltd. ("Company") will be held on December

21, 2017, at 10:00 am Israel Time, at the offices of the Company's Israeli counsel (Zysman, Aharoni, Gayer & Co.) at

"Beit Zion", 41-45 Rothschild Blvd., 8th Fl., Tel Aviv 65784,

Israel (the "Meeting"), for the following purposes:

Record Date and Right to Vote

Subject to the provisions of Israeli law

and the Company's Articles of Association ("Articles"), only shareholders of record as of the close of

trading on the Nasdaq Capital Market on November 21, 2017 ("Record Date") are entitled to attend and vote at

the Meeting and any adjournments or postponements thereof. They are also entitled to notice of the Meeting and to vote at the Meeting

if they held ordinary shares of the Company par value NIS 0.05 per share ("Ordinary Shares") through a bank,

broker or other nominee which was one of the Company's shareholders of record at the close of business on the Record Date.

shareholder whose Ordinary Shares are registered in his, her, or its favor with a member of a stock exchange and are included in

the Ordinary Shares which are registered in the register of shareholders of the Company under the name of such member of a stock

exchange, shall be required to prove ownership of such Ordinary Shares as of the Record Date by providing the Company, before the

time appointed for holding the Meeting, with proof of ownership, issued by a member of a stock exchange, or a copy of the shareholder's

Identification Card, passport, or Incorporation Certificate, if such shareholder is registered in the register of shareholders

Under the Articles, no business may be

transacted at any shareholders meeting unless a quorum is present when the meeting begins. The quorum required for a meeting

is the presence, in person or by proxy or by a voting deed, of at least two shareholders, holding in the aggregate at least one

third of the issued and outstanding Ordinary Shares as of the Record Date ("Quorum"). If within an hour

from the time appointed for holding a meeting a Quorum is not present, the meeting shall be dissolved and it shall stand adjourned

to the same day in the next week (or the business day following such day, if such day is not a business day) at the same time and

place and two shareholders then present at such adjourned meeting, in person or by proxy or by a voting deed, shall constitute

Abstentions are counted in determining

if a Quorum is present.

You can vote your shares by attending the

Meeting or by completing and signing a proxy card. Attached is the proxy card for the Meeting that is being solicited by our Board

of Directors. If you are voting by proxy, please follow the instructions on the proxy card. We encourage all shareholders to vote

by proxy, even if attending the Meeting.

We are mailing copies of this invitation

and the proxy card to our shareholders of record as of the Record Date, and we will solicit proxies primarily by mail and e-mail.

The original solicitation of proxies by mail and e-mail may be further supplemented by solicitation by telephone, mail, e-mail

and other means by certain of our officers, directors and employees (who will not receive additional compensation for these services).

We will bear the cost of external solicitors and of the solicitation of the proxy cards, including postage, printing and handling,

and will reimburse the reasonable expenses of brokerage firms and others for forwarding material to beneficial owners of our Ordinary

We will not be able to count a proxy card

unless we receive it, accompanied by a copy of (a) the shareholder's Identification Card, passport, or Incorporation Certificate

(if applicable), with respect to a shareholder who is registered in the register of shareholders of the Company, and (b) with proof

of ownership, as specified above, with respect to a shareholder whose shares are registered under the name of a member of a stock

exchange. We will not be able to count a proxy card unless we receive it at the offices of our Israeli legal counsel, Zysman, Aharoni,

Gayer & Co. at "Beit Zion", 41-45 Rothschild Blvd., 8th Fl., Tel

Aviv 65784, Israel, by December 21, 2017 at 6:00 am Israel time, (together with the foregoing proof of ownership). If mailing

your proxy card in the enclosed envelope, it must be received by our transfer agent, Vstock Transfer, LLC, at 18 Lafayette Place,

Woodmere, NY 11598 in the enclosed envelope, by 3:00 pm Eastern time on December 20, 2017 (an earlier deadline may apply for shares

held in street name, as may be indicated in the instructions provided to you with your proxy card).

If you sign and return the enclosed proxy

card, your shares will be voted as abstained of all of the proposed resolutions, whether or not you specifically indicate a "ABSTAIN"

vote, unless you specifically vote in favor or vote against a specific resolution.

The vote with respect to Item No. 6 of

the Meeting's agenda will not be counted without indication of non-existence of personal interest. See Items 5.a, in the

proxy card attached hereto.

On all matters considered at the Meeting,

abstentions will be treated as neither a vote "FOR" nor "AGAINST" the matter, although they will be counted

in determining if a Quorum is present.

Disclosure Regarding Compensation

New regulations under the Israeli Companies

Law require that the Company provides its shareholders with certain information about the compensation granted to the Company's

five most highly compensated officers during or with respect to the year ended December 31, 2016. Such information can be found

under Item 6.B. of the Company's Annual Report on Form 20-F for the year ended December 31, 2016 filed with the Securities

and Exchange Commission on February 24, 2017.

BIOBLAST PHARMA LTD.

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ANNUAL AND EXTRAORDINARY GENERAL MEETING

This Proxy Statement

is furnished to the holders of ordinary shares, NIS 0.05 nominal value per share ("Ordinary Shares"), of Bioblast

Pharma Ltd. ("Company") in connection with the solicitation by the Company's board of directors ("Board

of Directors") of proxies for use at the Annual and Extraordinary General Meeting of the Shareholders, or at any adjournment

thereof, pursuant to the accompanying Notice of Annual and Extraordinary General Meeting of Shareholders. The meeting will be held

on December 21, 2017, at 10:00 am Israel Time, at the offices of the Company's Israeli counsel (Zysman, Aharoni, Gayer &

Co.) at "Beit Zion", 41-45 Rothschild Blvd., 8th Fl., Tel Aviv 65784, Israel ("Meeting").

SOLICITATION OF PROXIES

The agenda for the Meeting

ITEM 1 - TO PRESENT THE FINANCIAL

STATEMENTS OF THE COMPANY FOR THE

FISCAL YEAR ENDED DECEMBER 31, 2016

The Company's financial

information for the year ended December 31, 2016 is available on the Company's website at the following address:

At the Meeting, the Company

will review the audited financial statements for the year ended December 31, 2016 and will answer appropriate questions relating

No vote will be required regarding this

ITEM 2 - RE-APPOINTMENT OF THE COMPANY'S

INDEPENDENT REGISTERED

PUBLIC ACCOUNTING FIRM

Audit Committee and Board of Directors recommend that the Company's shareholders re-appoint Kost, Forer, Gabbay & Kasierer,

a member of Ernst & Young Global, as the Company's independent registered public accounting firm until the next Annual

Meeting and to authorize the Board of Directors to determine its compensation for the fiscal year ending December 31, 2017. It

is proposed that the following resolution be adopted at the Meeting:

to re-appoint Kost Forer Gabbay & Kasierer (a member of Ernst & Young Global) as the Company's independent registered

public accounting firm until the next Annual Meeting and to authorize the Board of Directors to determine its compensation for

the fiscal year ending December 31, 2017."

The affirmative vote

of a majority of the shares voting on the matter is required to approve this resolution.

Board of Directors unanimously recommends that you vote "FOR" the proposal included in this Item.

ITEM 3 - RE-ELECTION OF DIRECTORS