Among the congenital anomalies of the coronary arteries, a left circumflex artery (LCX) defect is extremely rare. A 49-year-old man who developed an acute anterior infarction underwent coronary angiography, which revealed complete occlusion of the left main trunk, but the territory usually supplied by the LCX had been perfused by the superdominant right coronary artery. Treatment of the left main trunk by percutaneous coronary intervention produced a favorable result. Accurate evaluation of the principal vessels and the extent of compensatory perfusion is important when diagnosing ischemic heart disease accompanied by anomalous coronary arteries and for choosing the best treatment modality.