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 Wednesda

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

Wednesday, March 18, 2015, at 10:00 am (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,

Israel ("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 Global Market on February 18, 2015 ("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 Sunday, March 15, 2015 at 10:00 am Israel time, which is Sunday, March 15, 2015, at 3:00 am Eastern

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 Wednesday, March 18, 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

ITEM 1 - APPROVAL OF EMPLOYMENT TERMS

OF THE COMPANY'S NEW PRESIDENT AND CHIEF EXECUTIVE OFFICER, MR. COLIN FOSTER.

Pursuant to an amendment to the Israeli

Companies Law, all Israeli public companies, including companies whose shares are publicly-traded only outside of Israel, such

as the Company, are required to adopt a written compensation policy for their executives, which addresses certain items prescribed

by the Israeli Companies Law, within nine months of the consummation of their initial public offering. The Company is in the process

of adopting such compensation policy before the end of the nine months period. Until the adoption of such a policy, the approval

of the compensation of a chief executive officer who is also a director requires the Compensation Committee and Board of Directors

to take into account a list of considerations dictated under the Companies Law, which was done. In connection with the appointment

of Mr. Foster as President, Chief Executive Officer and a director of the Company, the Company's Compensation Committee and

the Board of Directors approved and recommend the shareholders to approve, Mr. Foster's employment terms substantially in

the form attached hereto as Exhibit A, and further determined that such terms are advisable and in the best interest

In making its recommendation, the Compensation

Committee and Board of Directors reviewed the list of considerations required to consider for the approval of the compensation

terms of a chief executive officer who is also a director, including (a) Mr. Foster's education, skills, expertise, professional

experience, and achievements; (b) Mr. Foster's position, responsibilities, and previous compensation arrangements; and (c)

the ratio between Mr. Foster's office and employment proposed terms and the salary of other Company employees and contractors,

in particular the ratio between the average salary and the median salary of such employees and the effect of such differences on

work relations within the Company; and

It is therefore proposed that the following

resolution be adopted at the meeting:

to approve the compensation terms of Mr. Colin Foster in connection with his office as President and Chief Executive Officer as

set forth in Exhibit A attached hereto."

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.1

The Company s board of directors

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

ITEM 2 -APPROVAL OF THE COMPENSATION

TERMS OF DR. DALIA MEGIDDO, IN HER NEW POSITION AS CHIEF DEVELOPMENT OFFICER.

As disclosed in the Company's prospectus

dated July 31, 2014, Dr. Megiddo, who is the Company's co-founder, has served as a Chief Executive Officer and director since

the Company's inception. Dr. Megiddo engagement with the Company was conducted in the form of a services agreement. On January

29, 2015, following the appointment of Mr. Colin Foster as the Company's President and Chief Executive Officer, Dr. Megiddo

was appointed as the Company's Chief Development Officer. Under her new position, Dr. Megiddo will be directly employed by