Schwartz-Jampel syndrome (SJS) is an autosomal recessive and rare disease that comprises facial and musculoskeletal deformity. The aims are to report two cases of SJS, demonstrate the film of surgical method, and review the literature in order to conclude if BTA injection or surgery could be the first and best choice of treatment of blepharospasm in SJS.

First case was a boy who was result of twin delivery whose sibling was completely normal. Second case was a girl who was the first child of her family. Both were born to a consanguineous marriage. They had Botulinum toxin A injection (twice in the first and ones in the second case) with no improvement.

Orbicularis oculi myectomy, partial corrugator muscle excision, levator tucking, and lateral tarsal strip procedure was performed on both cases. No complication and no recurrence of eyelid contraction were observed after 4 years and 6 months follow up in the first and second case, respectively.

BTA injection does not play a role in controlling the blepharospasm in patients with SJS and surgery should be considered the first line of treatment, based on our two case reports and literature review.