A case of bilateral renal artery stenosis and aneurysms in association with neurofibromatosis and hypertension is discussed and the literature is reviewed. In patients with neurofibromatosis and hypertension, the association of pheochromocytomas is well known. The association of renal vascular lesions must also be recognized. In children with neurofibromatosis and hypertension, renal artery stenosis is far more common than pheochromocytoma. In adults, aneurysms often accompany the renal artery stenosis. Laboratory studies, as well as most intravenous pyelograms, have been normal and, therefore, unreliable for screening. Angiography is essential in the investigation of hypertension in patients with neurofibromatosis.
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