Renal hypoperfusion from renal artery stenosis (RAS) activates the renin-angiotensin system, which in turn causes volume overload and hypertension. Atherosclerosis and fibromuscular dysplasia are the most common causes of renal artery stenosis. Recurrent flash pulmonary edema, also known as Pickering syndrome, is commonly associated with bilateral renal artery stenosis. There should be a high index of clinical suspicion for renal artery stenosis in the setting of recurrent flash pulmonary edema and severe hypertension in patients with atherosclerotic disease. Duplex ultrasonography is commonly recommended as the best initial test for the detection of renal artery stenosis. Computed tomography (CT) angiography (CTA) or magnetic resonance (MR) angiography (MRA) are useful diagnostic imaging studies for the detection of renal artery stenosis in patients where duplex ultrasonography is difficult. If duplex ultrasound, CTA, and MRA are indeterminate or pose a risk of significant renal impairment, renal angiography is useful for a definitive diagnosis of RAS. The focus of medical management for RAS relies on controlling renovascular hypertension and aggressive lifestyle modification with control of atherosclerotic disease risk factors. The restoration of renal artery patency by revascularization in the setting of RAS due to atherosclerosis may help in the management of hypertension and minimize renal dysfunction.