Abstract: Apert's syndrome is a rare congenital abnormality characterized by acrocephaly due to craniostosis and syndactyly of the hands and feet.The authors report a case of Apert's syndrome with a review of the literature.A 16-year-old girl consulted us about a displeased feeling in the mouth caused by abnormal tooth arrangement and masticatory disturbance.The patient showed cranio-facial anomalies, acrocephaly, maxillary hypolasia, exophtalmus, ocular hypertelorism and syndactyly of the hands and feet.