BIOBLAST PHARMA LTD. NOTICE OF EXTRAORDINARY GENERAL MEETING OF SHAREHOLDERS Notice is hereby given that an Extraordinary General Meeting of the Shareholders of BioBlast Pharma Ltd. (" Company ") will be held on

BIOBLAST PHARMA LTD.

NOTICE OF EXTRAORDINARY GENERAL MEETING

Notice is hereby given

that an Extraordinary General Meeting of the Shareholders of BioBlast Pharma Ltd. ("Company") will be held on

February 24, 2016, at 10:00 a.m. (Israel Time), at the offices of the Company's counsel, Zysman, Aharoni, Gayer & Co., at "Beit

Zion", 41-45 Rothschild Blvd., 8th Fl., Tel Aviv 6578401, Israel ("Meeting"), for the

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 Global Market on January 25, 2016 ("Record Date") are entitled to attend and vote at the Meeting

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

held ordinary shares of the Company par value NIS 0.01 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 our principal executive offices at 37 Dereh Menachem

Begin St., Tel Aviv 6522042, Israel or at our transfer agent, Vstock Transfer, LLC, at 18 Lafayette Place, Woodmere, NY 11598 in

the enclosed envelope, by February 24, 2016 at 6:00 a.m. Israel time, which is February 23, 2016, at 11:00 p.m. Eastern standard

If you sign and return the enclosed proxy

card, your shares will be voted in favor of all of the proposed resolutions, whether or not you specifically indicate a "FOR"

vote, unless you specifically abstain or vote against a specific resolution. 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.

BIOBLAST PHARMA LTD.

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EXTRAORDINARY GENERAL MEETING OF SHAREHOLDERS

This Proxy Statement

is furnished to the holders of ordinary shares, NIS 0.01 nominal value per share ("Ordinary Shares"), of BioBlast

Pharma Ltd. ("Company") in connection with the solicitation by the board of directors of proxies for use at

the Extraordinary General Meeting of Shareholders, or at any adjournment thereof, pursuant to the accompanying Notice of Extraordinary

General Meeting of Shareholders. The meeting will be held on February 24, 2016, at 10:00 a.m. (Israel Time), at the offices of

the Company's counsel, Zysman, Aharoni, Gayer & Co., at "Beit Zion", 41-45 Rothschild Blvd., 8th Fl., Tel Aviv 65784,

SOLICITATION OF PROXIES

The agenda for the Meeting

1 - APPROVAL OF SERVICE TERMS

in the Company's public filings and publications, Mr. Gilboa, is the Company's co-founder and served as the Company's

Chief Financial Officer ("CFO") until January 11, 2016, He also has served as a director since the Company's

inception. In connection with the termination of Mr. Gilboa's position as the Company's CFO, the parties agreed that

Mr. Gilboa will serve as a special advisor to the Chief Executive Officer of the Company, substantially under the same terms Mr.

Gilboa had as the Company's CFO, subject to a few changes that are being presented to the approval of the Company's

shareholders, such changes are presented in Exhibit A attached hereto.

engagement terms of Mr. Gilboa as set forth in Exhibit A shall not change the monthly cost to the Company.

proposed that the following resolution be adopted at the meeting:

to approve the amended terms of engagement with Mr. Gilboa, as set forth in Exhibit A.

The affirmative vote of a majority of the

shares voting on the matter is required to approve this resolution, providing either (i) included in such majority is at least

a majority of the shares of shareholders who are non-controlling shareholders and do not have a personal interest in the said resolution

(excluding for such purpose any abstentions disinterested majority); or (ii) the total number of shares of shareholders specified

in clause (i) who voted against this resolution does not exceed two percent (2%) of the voting rights in the Company.

The Company s board of directors

recommends that you vote "FOR" the proposal included in this Item.

The following is a summary of the main proposed

Note: The monthly cost of the engagement

with Mr. Gilboa under the new arrangement does not change compared with the terms approved previously by the Company's shareholders.

THIS PROXY IS SOLICITED

ON BEHALF OF THE BOARD OF DIRECTORS.

The undersigned hereby

appoints Mr. Fredric Price, Executive Chairman of the Board, Mr. Colin Foster, President and Chief Executive Officer, Mr. Bob Cook,

Chief Financial Officer and Mr. Dan Riemer, Controller, and each of them, agents and proxies of the undersigned, with full power

of substitution to each of them, to represent and to vote on behalf of the undersigned all the Ordinary Shares of BioBlast Pharma

Ltd. (the "Company") which the undersigned is entitled to vote at the Extraordinary General Meeting of

Shareholders (the "Extraordinary Meeting") to be held at the offices of the Company's counsel, Zysman, Aharoni,

Gayer & Co., at "Beit Zion", 41-45 Rothschild Blvd., 8th Fl., Tel Aviv, Israel, on February 24, 2016, at 10:00 a.m.

(Israel time), and at any adjournments or postponements thereof, upon the following matters, which are more fully described in

the Notice of Extraordinary General Meeting of Shareholders and Proxy Statement relating to the Extraordinary General Meeting.

This Proxy, when properly

executed, will be voted in the manner directed herein by the undersigned. If no direction is made with respect to any matter, this

Proxy will be voted FOR such matter. Any and all proxies heretofore given by the undersigned are hereby revoked.

to be signed on the reverse side)

EXTRAORDINARY GENERAL MEETING OF SHAREHOLDERS

AND RETURN PROMPTLY IN THE ENCLOSED EVNELOPE. PLEASE MARK YOUR VOTE IN BLUE OR BLACK INK AS SHOWN HERE