Amelogenesis imperfecta patients suffered common clinical problems of poor esthetics, teeth sensitivity, and loss of occlusal vertical dimension. Amelogenesis imperfecta is a group of inherited disorders primarily affecting dental enamel. Variants of amelogenesis imperfecta generally classified hypoplastic, hypocalcified, or hypomaturation types based on the primary enamel defects. The mildest problems were found in the pitted hypoplastic type whereas the most severe problems were encountered in the hypocalcified type amelogenesis imperfecta. Management stragies include composite resin veneer and jacket crowns for anterior teeth as well as steel crowns for posterior teeth. Knowledge of the clinical features and dental complications of each variants if amelogenesis impecta helps in the diagnosis of the condition and allows institution of early preventive measures. The objective of this paper is to provide a review of the current concepts of the wide spectrum of etiological factors involved in the pathogenesis of this significance clinical entity in the primary dentition.