An anomalous origin of the left coronary artery from the pulmonary artery is commonly associated with mitral valve insufficiency. Usually this is secondary to left ventricular dysfunction or papillary muscle ischemia. We describe the association of an anomalous left coronary artery from the pulmonary artery with structural abnormalities of the mitral valve (cleft mitral leaflet) in 2 children, both of whom were being investigated for primary mitral valve disease. Both underwent successful operations for coronary transfer and mitral valve repair.