From Jan. 1981 to Dec. 1991, we had treated 25 patients with congenital coronary art-eriovenous fistulas [CAVF] in Seoul National University Hospital. A retrospective review was made to delineate the course and the management of CAVF and to clarify the role of surgical treatment. Fifteen patients were male and 10 were female with The mean age of 17.4 years[from 3 months to 58 years]. The most frequent symptom was dyspnea on exertion[56%]. Other symptoms were angina and palpitation. Sixty-eight percent of the patients were symptomatic. Fifty-three percent of patients less than 20 years old were symptomatic and 100% of patients over 20 years old were symptomatic. Three patients had multiple CAVFs. The fistula drained into the right ventricle in 13, pulmonary artery in 9, left ventricle in 4, right atrium in 2, and left atrium in 1. Thirteen patients had other associated cardiac lesions. The mean pulmonary-to-systemic blood flow[Qp /Qs] in the isolate CAVF group was 2.19. All patients were operated on to correct the fistulas and other associated cardiac lesions. All patients were followed from 1 month to 11 years without late death. Postoperative complication rate was 24% -significant arrhythmia [3], recurred CAVF[1], psychosis[1], pneumonia [1]. Symptomatic improvement was evident postoperatively. Below 20 years old, 94% of patients were asymptomatic, but above 20 years old, symptoms persisted in 25%. In summary, early elective repair of CAVF is indicated in all patients because of higher complication rate and frequent persistent symptoms in older patients.