The causes of dental pigmentation are diverse. It can be classified in intrinsic or extrinsic depending on the origin and location of the stain in the affected tooth. This report presents an unusual case of dental pigmentation and enamel loss where the diagnosis of its origin revealed an underlying systemic pathology, unknown to the patient, which could have affected the development of the pigmentation. Keywords: dental pigmentation, diagnosis, discoloration, hemochromatosis, iron overload, tooth wear