A forty-eight-year-old woman was admitted because of substernal chest pain, profuse diaphoresis, vomiting and palpitation. Electrocardiography suggested acute coronary syndrome. However, the emergency coronary angiographic findings were normal. Periodic fluctuations in her blood pressure raised a clinical suspicion of pheochromocytoma. A subsequent computed tomography scan revealed a single left adrenal mass (8.5×8×6cm). Elevated 24-hour urine levels of vanillylmandelic acid and catecholamines supported the diagnosis of pheochromocytoma. After therapy with adrenergic blockade, a repeat ECG revealed near complete resolution of the ischemic changes. Her symptoms, diabetes and hypertension resolved after a left adrenalectomy. This case illustrates that pheochromocytoma should be included in the differential diagnosis of acute coronary syndrome.