Apert described acrocephalosyndactyly as a clinical entity in 1906. At first he included a patient with craniofacial deformities combined with syndactyly of both the upper and lower extremities. This is a rare congenital disturbance in the growth of bone and soft tissue affecting principally the head, the hands and the feet. More than 200 cases have been reported in the world literature, and three cases in Korea. Authors experienced one case of the classic pattern of Apert's syndrome and treated surgically for syndsctyly.