Amelogenesis imperfecta (AI) is a heterogeneous group of hereditary disorders primarily affecting dental enamel. This paper describes the clinico-radiological features of a 12-year-old female patient who was born of consanguineous parents and presented with smooth yellow discolored and misshapen teeth, small crowns, delayed eruption of permanent teeth, retained deciduous teeth, open contact areas between teeth, mild gingival involvement and anterior open bite. Radiograph revealed intrapulpal calcifications, enlarged dental sac surrounding unerupted molars and uniform radiodensity of enamel and dentin. Similar condition was observed in her 9-year-old brother. None of the other family members showed any evidence of AI. The diagnosis of smooth hypoplastic Amelogenesis imperfecta was confirmed. This case is first of its kind presenting the association of smooth hypoplastic Amelogenesis imperfecta type with anterior open bite and intrapulpal calcifications.
Key words: Amelogenesis imperfecta, Smooth hypoplastic type, Anterior open bite.