Renal artery stenosis (RAS) is a common form of peripheral arterial disease. The most common cause of RAS is atherosclerosis. It is predominantly unilateral. The pathophysiologic mechanism stems from renal underperfusion resulting in the activation of the renin- angiotensin-aldosterone pathway. Even though the majority of patients with RAS are asymptomatic, it can clinically present with hypertension, nephropathy and congestive heart failure. This progressive disease can lead to resistant hypertension and end stage kidney failure. Screening patients for RAS with either Doppler ultrasonography, computed tomographic angiography, or magnetic resonance angiography is preferred. Adequate blood pressure control, goal-directed lipid-lowering therapy, smoking cessation, and other preventive measures form the foundation of management of patients with RAS. Catheter-based percutaneous revascularization with angioplasty and stenting showed modest clinical benefit for patients in small retrospective studies, but data from randomized clinical trials failed to confirm these beneficial results. The current ongoing Cardiovascular Outcomes in Renal Atherosclerotic Lesions (CORAL) trial may provide more concrete data regarding the role of stenting in RAS. Surgical revascularization is considered only if catheter-based revascularization is unsuitable or unsuccessful. The American College of Cardiology/American Heart Association guidelines on evaluation and management of patients with RAS provide the framework for determining individualized assessment and treatment plans for patients with RAS.