The case of a 12-year-old boy with neurofibromatosis, kyphoscoliosis, and hypertension is reported. The association of neurofibromatosis and kyphoscoliosis is well known. There are no reports on the association of neurofibromatosis and hypertension in the orthopaedic literature. The cause was found to be intrarenal vascular abnormalities which are characteristic of neurofibromatosis. Other possible causes of hypertension in this disease are discussed. This patient was successfully treated by a partial nephrectomy.