Atheromatous renovascular disease (ARVD) is increasingly suspected and diagnosed, and it commonly presents to several different clinical specialties. In this review, the epidemiology, risk factors, comorbid disease associations, natural history, and prognosis of ARVD is described. Atheromatous renovascular disease is strongly associated with macrovascular pathology in other important vascular beds, especially the coronary, aortoiliac and iliofemoral circulations, and also with structural and functional heart disease. These clinicopathologic relationships contribute to the high morbidity and mortality associated with the condition. Understanding of the natural history of renal artery stenosis may enable intensified treatment strategies to reduce associated risk and improve patient prognosis.