Congenital erythropoietic porphyria is a rare condition resulting from an inborn error in prophyrin metabolism. This deficiency leads to hemolytic anemia, photosensitivity, blistering of the skin, and deposition of red-brown pigments in the bones and teeth. The literature regarding the dental aspects of this disorder is briefly reviewed and the preventive, restorative, and esthetic dental management of a 4-year-old child with congenital erythropoietic porphyria is described.