Amelogenesis imperfecta (AI) is a disorder group of hereditary development that affects the dental enamel structure which is marked by clinical alterations without association with systemic abnormalities and diseases. The AI trait can be transmitted by either autosomal dominant, autosomal recessive, or X–linked modes of inheritance. Genes implicated in autosomal forms are genes encoding enamel matrix proteins, namely: enamelin and ameloblastin, tuftelin, MMP–20 and kallikrein – 4. Here we present a case of 5 year old girl having amelogenesis imperfecta affecting the deciduous dentition which was diagnosed on the basis of classical clinical and radiographic features.