Neurofibromatosis (NF1) is a relatively common autosomal dominant disorder. Secondary causes of hypertension, such as renovascular disease, coarctation of the abdominal aorta or phaeochromocytoma, may be identified in up to 1% of patients with NF1. Usually, renal angiography, which is always used to confirm the diagnosis of renovascular hypertension, reveals stenoses and rarely bilateral or unilateral renal artery aneurysms. We present the first description of a percutaneous transluminal renal angioplasty performed in an adult female patient with NF1, severe hypertensive disease and renal artery aneurysms, in order to restore renal artery anatomy and treat renovascular hypertension.