We report a rare case of congenital coronary artery anomaly with recurrent chest pain. A 44-year-old Taiwanese female patient presented with exertional chest pain that had lasted for 2 years. An electrocardiogram showed right axis deviation and an rS pattern in leads I and aVL, and an exercise stress test was inconclusive. A thallium-201 myocardial perfusion study revealed perfusion defects in the septal and inferior walls which normalized in the delayed imaging. Coronary angiography revealed the absence of a left circumflex coronary artery and a superdominant right coronary artery with terminal branches supplying the left ventricular inferior and posterolateral walls. An aortogram revealed no evidence of the existence of a left circumflex coronary artery. Administration of nitrates and calcium antagonists could not ameliorate her symptoms, but she remained stable during ordinary activity. In young premenopausal female patients who present with symptoms suggestive of angina pectoris and a positive stress test, congenital anomaly of the coronary artery should be considered and an angiographic study should be performed.