Pheochromocytomas have been described to be associated with rare vascular abnormalities, most common of them being renal artery stenosis. A 45-year-old woman was admitted to our hospital with complaints of headache, sweating, anxiety, dizziness, nausea, vomiting and severe hypertension. Hypertension was confirmed to result from both excess catecholamine production and hyperreninemia of left kidney. The technical images (abdominal CT and renal arteriography) revealed the presence of a left adrenal pheochromocytoma and stenosis of the renal artery. Surgical removal of pheochromocytoma and correction of renal artery stenosis restored the postoperative plasma catecholamine, renin and blood pressure to normal. To our belief, this is the first such case report from India citing this rare association. We conclude that when the two diseases occur simultaneously, both must be diagnosed accurately and treated in a different manner. We also hereby review the existing literature.