[Amelogenesis imperfecta. Description of a clinical case]

A family of five was examined. Four of them presented with enamel alteration including changes in colour and loss of enamel surface. A genetic study was undertaken which revealed an autosomal dominant inheritance with complete penetration and variable expressivity. This is reflected in shape, number, extension and depth of the affected areas. Amelogénesis Imperfecta type IV was diagnosed using Shields classification.