Dentinogenesis imperfecta is a congenital dentin dysplasia that occurs either isolated or associated with a genetic disorder known as osteogenesis imperfecta. Dentinogenesis imperfecta is inherited in an autosomal dominant pattern. Clinically the teeth color of both dentitions varies from brown to a translucent gray with an opalescent sheen. Shields et al. (1973) proposed a classification of Dentinogenesis imperfecta into three types: type I, associated with osteogenesis imperfecta; type II, hereditary opalescent dentin; type III Brandywine-type. The phenotypes of Dentinogenesis imperfecta are described in regard to their genetic defects, pathology, radiology and histopathology as well as their dental treatment.