Oculofaciocardiodental (OFCD) syndrome is an extremely rare condition with ocular, facial, cardiac, and dental abnormalities. This is a X-linked rare congenital anomaly, with incidence of less than 1 in 1 million people and it might be lethal in males. It is characterized by heterogeneous clinical features such as dental radiculomegaly, congenital cataract, facial dymorphism and congenital heart disease. Isolation and diagnosis of this syndrome is hard for the medical specialists whereas itcan easily be diagnosed by an orthodontist or general dentist as it presents unique and specific findings in dental and skeletal structures