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

BIOBLAST PHARMA LTD.

NOTICE OF ANNUAL GENERAL MEETING OF THE

Notice is hereby given that an Annual General

Meeting of the Shareholders of BioBlast Pharma Ltd. ("Company") will be held on August 9, 2016, 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 (the "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 July 11, 2016 ("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 0.01 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 August 9, 2016 at 6:00 am Israel time, which is August 8, 2016 at 11:00 pm Eastern time.

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 Items No. 6, 7

and 8 of the Meeting's agenda will not be counted without indication of non-existence of personal interest. See Items 5.a,

6.a, and 7.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 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, 2015. 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, 2015 filed with the Securities

and Exchange Commission on March 29, 2016.

BIOBLAST PHARMA LTD.

ANNUAL AND EXTRAORDINARY GENERAL MEETING

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 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 General Meeting of Shareholders. The meeting will be held on August 9, 2016, 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").

SOLICITATION OF PROXIES

The agenda for the Meeting is as follows:

ITEM 1 - TO PRESENT THE FINANCIAL

STATEMENTS OF THE COMPANY FOR THE FISCAL YEAR ENDED DECEMBER 31, 2015

The Company's financial

information for the year ended December 31, 2015 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, 2015 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

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, 2016. 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, 2016."

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.

1 The contents of the Company's

website do not form a part of, and are not incorporated by reference into, this proxy statement.

ITEM 3 - RE-ELECTION OF DIRECTORS

Articles of Association provide that other than external directors (who are elected and serve in office in accordance with the

provisions of the Companies Law), the directors in the Company shall be elected at an Annual Meeting and shall serve in their office

until the next Annual Meeting, or until they cease to serve in their office in accordance with the provisions of the Articles of

Association or applicable law, whichever is the earlier. Mr. Gili Cohen, our sole external director, is not standing for re-election

as his election is valid for three years, from the date of his election as an external director on October 30, 2014.

Companies Law, all director candidates have declared in writing that they possess the requisite skills and expertise, as well as