Oculo-facio-cardio-dental (OFCD) syndrome is an X-linked dominant syndrome characterized by congenital cataract, microphtalmia, secondary glaucoma, long narrow face, high nasal bridge, pointed nose with cartilages separated at the tip, cleft palate, atrial septal defect (ASD), ventricular septal defect (VSD), radiculomegaly, delayed eruption, oligodontia, and persistence of primary teeth. The aim of this report is to indicate the importance of early diagnosis and management of oculo-facio-cardio-dental syndrome.