We present a 25-year-old female patient with Bland-White-Garland syndrome (anomalous origin of the left coronary artery from the pulmonary artery) who underwent mitral valve replacement (MVR) and coronary artery bypass grafting. She had previously undergone MVR and had been treated by the Takeuchi procedure for mitral valve regurgitation and a coronary artery anomaly, respectively, at 17 years of age. She was hospitalized with shortness of breath within 7 years of surgical intervention. Swan-Ganz catheterization revealed mild pulmonary hypertension and elevated mean pulmonary wedge pressure. Echocardiography revealed marked severe calcification of the bio valve and moderate mitral valve regurgitation. Coronary angiography revealed retrograde collateral blood flow from the right coronary artery to the left coronary artery, but intrapulmonary tunnel was not visualized by pulmonary arteriography. We performed MVR with a mechanical valve and coronary artery bypass grafting between the left internal thoracic artery and the left anterior descending artery. The postoperative course was unremarkable, and no complaints were reported during follow-up at the outpatient clinic.