Apert syndrome, or acrocephalosyndactyly is a complex of associated malformations, which are craniosynostosis and symmetric complex syndactyly of the hands and feet. The syndrome was first described by Apert in 1906. Review of the worlds literature disclosed about 200 similar cases and 9 Cases in Korea. Authors experienced one case of Apert syndrome and treated surgically for acro-syndactyly and polydactyly of both hands and feet. The case was compared with the other previously reported cases in Korea.