Congenital fistula between a coronary artery and the left cardiac ventricle are extremely rare. Since Blakeway (1918) first described this type of anomaly, only 72 cases have been reported up to the present, within our knowledge. A surgical correction of congenital fistula of the right coronary artery to left ventricle in a 36 year-old woman who suffered from easy fatigability is reported. This lesion was deduced after echocardiography identified a dilated right coronary artery and fluttering of the posterior cusp of the mitral valve throughout the diastole, the diagnosis was confirmed by retrograde aortography and coronary angiography. The fistula was closed by Symbas's operation under cardiopulmonary bypass with cardiac arrest and mild hypothermia. Postoperative recovery was uneventful. Coronary angiography one month after the operation revealed that the right coronary artery was still dilated with thrombus; there were no signs of myocardial ischemia or infarction. This diagnosis and successful treatment were based on careful study of the documentation of the 72 previously reported cases.