Hallermann-Streiff syndrome (HSS) is a rare oculomandibulofacial discephaly with hypotrichosis that occurs as a sporadic mutation. It is characterized by abnormal findings especially in head and face. Dental anomalies occur in 50-80% of the patients. In this case report, facial-oral findings and the existing dental anomalies of the syndrome in a 6-year-old male patient diagnosed with HSS were identified. Dental rehabilitation and a 12-month follow-up of the patient were reported. Each case presented with this rare syndrome may contribute to the literature to determine the prognosis of the disease and to take protective and preventive measures.

Keywords: Dental anomalies; Hallermann-Streiff syndrome; Tooth agenesis; Primary dentition; Nance appliance; Pediatric dentistry.