A 53-year-old female presented with symptoms of severe chest and back pain associated with oliguria. The patient had a history of exertional dyspnea since the age of 20, and easy fatigability since the age of 27. At the age of 41, she noted marked exacerbation of these symptoms after suffering from a cold and was ultimately diagnosed as having Bland-White-Garland (BWG) syndrome with mitral valve regurgitation. The patient then underwent re-implantation of an anomalous left coronary artery from the pulmonary artery to the posterolateral wall of the aorta. Eleven years later, she re-presented with symptoms of angina and congestive heart failure. Coronary angiography was subsequently performed and a total occlusion of the right coronary artery with probable thrombus was revealed. The right coronary artery was filled via collaterals from the implanted left coronary artery. Mitral regurgitation was noted during angiography. The patient underwent aorto-coronary artery bypass grafting of the right coronary artery and concomitant mitral valve replacement. Her postoperative condition remained excellent.