Oculo-facio-cardio-dental (OFCD) syndrome is an extremely rare condition with ocular, facial, cardiac, and dental abnormalities. It is often unrecognized by many medical and dental professionals. Only 17 cases have been documented to date. Because all reported patients have been female, it has been suggested that OFCD syndrome is an X-linked dominant trait. Isolation and diagnosis of this syndrome is hard for the medical specialists. Only unique and specific findings are observed in dental and skeletal structures, which can easily be diagnosed by an orthodontist or general dentist. Therefore, it was the aim of this study to present a new case of this syndrome and to evaluate it from an orthodontic perspective in order to call the attention of orthodontists to this rare anomaly.