Renovascular hypertension (RVH) represents a secondary and potentially remediable form of hypertension. Elevated blood pressure is only one of a broad array of pathophysiologic consequences that are associated with decreased renal perfusion. Our ability to accurately and noninvasively detect stenotic lesions within the renal artery is growing. However, functional assessment of renal parenchyma and hemodynamic significance of renal artery lesions is still limited. Advances in endovascular techniques spurred interest in the concept of ischemic nephropathy and the effect of renal artery revascularization on renal function. Despite the relative frequency of atherosclerotic renal artery stenosis (ARAS), there currently is no consensus on the most appropriate therapy. In this article, we focus on the two most common causes of RVH, ARAS and fibromuscular dysplasia. We discuss the therapeutic strategies, disease mechanisms, clinical findings, evolving trends, and developments.