We report a 50-year-old female case of mitral stenosis with congenital coronary artery fistula communicating the left anterior descending artery to pulmonary artery. In reviewing the literature, mitral stenosis associated with coronary artery fistula is rare. The case was initially treated medically for congestive heart failure. The electrocardiogram revealed severe myocardial ischemia and no obvious etiology was found clinically. The coronary angiogram demonstrated the diagnosis of coronary artery fistula. Myocardial ischemia improved markedly after surgical correction of the valvular disease and the fistula. The patient continued to do well during 2 years and 10 months follow up. The concomitant mitral stenosis masked the symptoms of coronary artery fistula, and made us fall to diagnose the condition initially. Valvular heart disease associated with severe myocardial ischemia without obvious atherosclerotic stenosis of coronary artery reminded us of the possibility of coronary artery fistula, though it has rarely been reported.