Background: Congenital clasped thumb is a deficiency of active extension of the thumb with flexion and adduction deformity after the age of 3 months. In distal arthrogryposis, congenital clasped thumb is characterized by global metacarpo-phalangeal (MCP) joint instability; severe narrow 1st web space and palmar contracture. Objective: The present study aimed to describe patients' characteristics, and the results of treatment of congenital clasped thumb in distal arthrogryposis. Patients and methods: A total of 24 congenital clasped thumbs in 14 patients (9 boys and 5 girls) underwent release of tight first web space structures, Ghani flap, and MCP joint chondrodesis with or without flexor pollicis longus lengthening. There were 10 bilateral and 4 unilateral cases. The mean age of the patients was 4 years old (ranging from 3 to 5). Results: According to the parent parents of the participants, all patients were fulfilled with the thumb function and appearance. Union of the chondrodesis was achieved in 21 thumbs. There was statistically significant improvement of thumb abduction, rotation and opposition. Conclusions: The 1st web space contracture release, Ghani flap and MCP chondrodesis is a good surgical option for management of congenital clasped thumb in distal arthrogryposis.