BIOBLAST PHARMA LTD. NOTICE OF EXTRAORDINARY GENERAL MEETING OF THE 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 September 18, 2017,

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 following purpose:

Record Date and Right to Vote

Subject to the provisions of Israeli law

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

of the close of trading on the Nasdaq Capital Market on August 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 0.01 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

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 the Company's counsel (Zysman,

Aharoni, Gayer & Co.) at "Beit Zion", 41-45 Rothschild Blvd., 8th Fl., Tel Aviv, Israel, or at our transfer agent,

Vstock Transfer, LLC, at 18 Lafayette Place, Woodmere, NY 11598 in the enclosed envelope, by September 18, 2017 at 6:00 am Israel

time, which is September 17, 2017 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 "FOR" or "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.

order of the Board of Directors,

of the Board of Directors

BIOBLAST PHARMA LTD.

EXTRAORDINARY GENERAL MEETING OF THE

This Proxy Statement is furnished to the

holders of ordinary shares, New Israeli Shekel ("NIS") 0.01 par value per share (the "Ordinary Shares"),

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

directors (the "Board of Directors") of proxies for use at the extraordinary general meeting of the shareholders,

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

will be held on September 18, 2017, 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").

SOLICITATION OF PROXIES

The agenda for the Meeting

a reverse split of the Company's share capital at the ratio of five to one and to amend the Company's Amended and Restated

Articles of Association accordingly.

the Company received a staff deficiency letter from NASDAQ notifying the Company that for the past 30 consecutive business days,

the closing bid price per share of the Company's Ordinary Shares had been below the $1.00 minimum bid price requirement for

continued listing on the NASDAQ Capital Market, as required by NASDAQ Listing Rule 5550(a)(2) (the "Bid Price Rule").

NASDAQ provided the Company with 180 calendar days, or until October 9, 2017, to regain compliance with the Bid Price Rule. To

regain compliance with the Bid Price Rule, the closing bid price of the Ordinary Shares must meet or exceed $1.00 per share for

a minimum of 10 consecutive business days during the 180 day grace period. In general, applicable NASDAQ rules provide that upon

meeting certain NASDAQ listing requirements, a company may be eligible for an additional grace period of 180 calendar days. However,

the Company does not meet all such NASDAQ listing requirements and therefore is not eligible for such additional grace period.

If the Company fails to

regain compliance with the Bid Price Rule and its Ordinary Shares are delisted, trading in the Ordinary Shares would be expected

to be conducted on the Pink Sheets. The Pink Sheets is generally considered to be a less efficient market than the NASDAQ Capital

Market, and the stock price, as well as the liquidity of the Ordinary Shares, could be adversely affected as a result. In order

to regain compliance with the Bid Price Rule, our Board of Directors recommends that the Company's shareholders effect a

reverse split of the Company's share capital at the ratio of five to one, as detailed below.

It is proposed that the

following resolution be adopted at the Meeting:

to approve a reverse split of the Company's share capital at the ratio of five to one, so each five ordinary shares, par value

NIS 0.01 per share, shall be consolidated into one ordinary share, par value NIS 0.05 (the "Reverse Split").

the Board of directors of the Company is authorized to cause any shareholders (and any other security holders) who otherwise would

be entitled to receive fractional shares as a result of the Reverse Split, because they hold a number of shares not evenly divisible

by five, to be automatically entitled to receive an additional fraction of a share to round up to the next whole share.

RESOLVED, to approve

and to amend the Company's Amended and Restated Articles of Association to reflect the Reverse Split, such that as a result,

the authorized share capital of the Company shall be NIS 500,000 divided into 10,000,000 ordinary shares, par value NIS 0.05 per

share, and Article 2.1.1 of the Company's Amended and Restated Articles of Association will be replaced in its entirety with

The registered capital

of the Company is NIS 500,000 divided into 10,000,000 ordinary shares with a par value of NIS 0.05 each."

THIS PROXY IS SOLICITED

ON BEHALF OF THE BOARD OF DIRECTORS.

The undersigned hereby

appoints Mr. Fredric Price, Executive Chairman of the Board of Directors and Chief Executive Officer, Mr. Chaime Orlev, and Mr.

Oren Elmaliah, and each of them, as 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, New Israeli Shekel ("NIS") 0.01