Amelogenesis imperfecta is a group of hereditary developmental defect in enamel formation either in quality and/or quantity which affects both deciduous and permanent teeth. It may be hypoplastic, hypomaturation, hypocalcified type. Hypomaturation-hypoplastic AI with taurodontism is a rare variant. AI has been associated with different dental anomalies including discoloured teeth, taurodontism, congenitally missing teeth, microdontia, pulpal calcification, hypercementosis, crown and root resorption. This is a case report of Hypomaturation AI with taurodontism in siblings born to parents with consanguineous marriage and congenitally missing tooth in one of the sibling.
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