Dentinogenesis imperfecta (DGI) is a disease inherited in an autosomal dominant mode characterized by abnormal dentin structure affecting either the primary or both the primary and secondary dentitions. Early diagnosis and treatment of DGI are recommended, as it may prevent or intercept deterioration of the teeth and occlusion and improve esthetics. The article is to briefly review the manifestations, diagnostic methods and early treatment strategy of DGI, and presents a case report of a family affected with DGI in siblings.