Over a 25-year period, 58 patients underwent evaluation or surgical treatment of coronary artery fistulas, or both, at our institution. Twenty-one patients had major associated cardiac defects, either congenital or acquired, and 9 had multiple fistulas. Fistulas to the left heart were unusual. Exertional angina and dyspnea were the most common presenting symptoms, but most young patients were asymptomatic. Seventy percent had audible heart murmurs. No operative deaths occurred in patients with isolated fistulas. Coronary artery fistulas cause symptoms due to "coronary steal" with resulting myocardial ischemia and, more rarely, marked left-to-right or left-to-left shunting. Definitive surgical correction is safe and effective, with good long-term results.