We report a case of oculodentodigital dysplasia in an 18-year old man. This symptom complex is characterized by : ocular anomalies which include epicanthus, microphthalmia and/or microcornea ; face anomalies including a thin nose with hypoplastic alae ; digital disfigurations including syndactyly and camptodactyly of the fourth and fifth fingers, and hypoplasia of the middle phalanges ; and severe hypoplasia of enamel of all the teeth. The patient showed all of the typical findings of oculodentodigital dysplasia but in addition he shows congenital nystagmus, dizziness on neck extension, osteopetrosis of skull, anomalous odontoid process, conductive hearing loss and anomalous auricle and ossicle.