Genetic disorders of the dentin are known for several years, and reviews have been published mainly in the form of case reports. Dentinogenesis imperfecta (DI) is a disease characterized by an abnormal formation and abnormal structure of the dentin, generally affecting both primary and permanent dentition. The only epidemiological data available were published in 1975 by Witkop, who estimated the incidence of dentinogenesis imperfecta to be between 1/6000 and 1/8000. We present a case of DI III with an unusual presentation of rhizomegaly, dilacerations of the root, and taurodontism, seldom reported in the English literature for any genetic disorder of dentin. Clinical management is challenging in such cases, as the literature mentions that only an early diagnosis and early treatment with periodic follow-up can help improve the quality of life. Psychological and genetic counseling along with a multidisciplinary approach is mandatory for the cases such as the one presented here. Patient education and motivation had a paramount role in making the patient accept the treatment.

Dentinogenesis Imperfecta is a rare hereditary dentin developmental disorder that affects both primary and permanent dentition. It is characterized by discolored and translucent teeth ranging from gray to brownish-blue/amber. The enamel may split readily from the dentin when subjected to occlusal stress. Radiographically, there is evidence of cervical construction, short root, and pulp chambers, whereas the root canals are smaller than normal or completely obliterated. Here, the author presents the case of a 28-year-old male with generalized enamel hypoplasia and isolated distinctly translucent second premolars in three quadrants along with root changes. Unlike the classical representation of generalized involvement of the teeth, this case differs and needs to be documented. A thorough history, careful clinical, and radiographic observation are the key to early diagnosis and management of such a rare entity.