With approximately 150 cases reported to date, congenital erythropoietic porphyria (CEP) is an extremely rare, autosomal recessively inherited condition. In few cases this deficiency leads to deposition of red-brown pigments in the teeth. This case report described the microscopic study of the extracted tooth sample along with preventive, restorative dental management and oral health counseling in a rare case of Congenital Erythropoietic Porphyria.
Key words: Erythropoietic porphyria, oral health, pigmentation.