Hallermann-Streiff Syndrome (HSS) is a rare disorder characterized primarily by head and face abnormalities, with dental abnormalities also present in 50-80 percent of cases. The first description seems to have been made by Aubry in 1893. HSS was first described completely in 1948 by Hallermann, and then in 1950 by Streiff. We report a 4-year-old girl with HSS, presenting oro-dental characteristics and describe the treatment modalities. An interdisciplinary approach including early preventive-care programmes, detailed oral hygiene instructions, dietary recommendations, counselling of the parents and regular dental visits are the essential procedures.