Neurofibromatosis type 1 (NF-1) is an autosomal dominant, hereditary, neurocutaneous syndrome that may, primarily or secondarily, affect different organs or systems of the body including the cardiovascular system. The most common vascular abnormality in patients with NF-1 is renal artery stenosis. Here we report the case of a middle-aged gentleman who presented at Sultan Qaboos University Hospital, Oman, with end stage renal disease and severe hypertension and was diagnosed to have NF-1 with bilateral renal artery stenosis. He was started on renal replacement therapy.