This clinical case illustrates the diagnosis of a secondary cause of hypertension in a patient with Von Recklinghausen's disease. The hypertension resulted from a complex malformation of the right renal artery and dysplasic stenosis of the left inferior polar artery treated successfully by simple angioplasty. This case illustrates the high proportion of vascular (renal artery dysplasia, coarctation of the aorta) and endocrine (pheochromocytoma) causes of hypertension in patients with neurofibromatosis.