Renal artery stenosis (RAS) is an important cause of renal insufficiency, refractory hypertension, and cardiac destabilization syndromes with increased cardiovascular events and mortality. Renovascular hypertension (RVH) occurs in 1–2% of the overall hypertensive population but the prevalence may be as high as 10% in patients with resistant hypertension and even higher in patients with accelerated or malignant hypertension. Cardiovascular challenges in managing RAS are RVH, ischemic nephropathy and salvaging renal function. Interrelated renovascular syndromes associated with RAS can be broadly classified as anatomic RAS, renin dependent hypertension, essential hypertension, reversible renal ischemic dysfunction and irreversible ischemic nephropathy. Pathophysiology of renovascular hypertension is discussed. Long-term management of the patient with RVH represents a balance between pharmacologic management of BP and cardiovascular risk and optimal timing of renal revascularization. Several factors favor medical therapy and surveillance in RAS. In addition, RAS and refractory hypertension, revascularization, and endovascular renal angioplasty and stenting are discussed.