We report an exceptional case of ischemic heart disease due to the origin of the left coronary circumflex artery from the pulmonary artery in a 50-year-old woman. She had undergone surgery for aortic coarctation when she was 16 years old. This abnormality was associated with other congenital defects such as tunnel subaortic stenosis, small aortic valve annulus, numerous left ventricular false tendons, and aortic bicuspid valve. Cardiac surgery verified the origin of the left circumflex from the pulmonary artery. The left internal mammary artery was positioned on the obtuse marginal coronary branch. Her clinical state was moderately improved 3 months after surgery.