Renal artery stenosis (RAS) is a cause of hypertension and ischemic nephropathy. The incidence of this disorder is probably less than 1% in patients with mild hypertension, but rises to as high as 10 to 40% in patients with acute, severe or refractory hypertension. Significant RAS can be caused by atheromatous plaques, or due to fibromuscular dysplasia (FMD). Atherosclerotic lesions are present in almost 7% of adults older than 65 years and up to 50% of patients presenting with diffuse atherosclerotic disease. In contrast to atherosclerosis, FMD most often affects women under the age of 50 and typically involves the distal main renal artery or the intrarenal branches. The optimal treatment for RAS is not yet established. Based on recent trials, we reviewed the literature on pharmacological and endovascular treatment of atherosclerotic RAS and ischemic nephropathy.