Besides the more frequent aspects, an unusual manifestation of renal osteodystrophy occurred in a 19-year-old man on regular hemodialysis treatment. About 6 years after the start of hemodialysis, the patient, who had severe osteodystrophy, presented a brown tumor, localized to the jaw, which caused a serious deformity of the face. Pharmacological treatment for hyperparathyroidism did not reduce the volume of the mass. Only total surgical removal gave long-lasting success. Brown tumor is an unusual but serious complication of renal bone disease which can be treated successfully by surgical removal of the tumoral mass and by pharmacological treatment for hyperparathyroidism.