Anomalous left coronary artery arising from the pulmonary artery (ALCAPA) has been generally reported as an isolated lesion that is also called Bland-White-Garland syndrome. Herein we report a case of ALCAPA syndrome with an atrial septal defect in a 68-year-old woman. She had been asymptomatic until the age of 68. Echocardiographic examination revealed atrial septal defect, and coronary angiography showed that the left main coronary artery originated from the pulmonary artery and intensive collateral connections between the right and left coronary artery. In this case, it would appear that ALCAPA is associated with atrial septal defect.