Congenital arteriovenous coronary fistulae are a rare condition of a direct communication between a coronary artery and one of the cardiac chambers, the coronary sinus, the superior vena cava or the pulmonary artery. In most instances the diagnosis is made during heart catheterization for coronary or congenital heart disease. Whether congenital coronary artery fistulae should be treated by transcatheter intervention or surgery and in which patients fistula closure should be performed is controversial. This report summarizes our experience of the surgical treatment of congenital arteriovenous coronary fistulae in 14 patients at the Deutsches Herzzentrum Berlin between March 1988 and April 1997. There were seven females and seven males aged from 3 to 67 years (mean 47 years). We analyzed the symptomatic status (NYHA class) preoperatively and in the late outcome, the preoperative angiographic data and the surgical techniques. The right coronary artery was affected in six, the left in six, and both arteries in two cases. The fistulae drained into the pulmonary artery in eight cases, into the superior vena cava and into the right atrium in two cases, and into the right ventricle and into the coronary sinus once. Fistula closure was unsuccessfully attempted interventionally in two patients and surgically in one patient in another institution. Twelve of the patients exhibited additional cardiac disease requiring surgery: seven cases presented additional coronary artery disease, one mitral valve disease, one persistent ductus arteriosus, one an aneurysm of the right coronary artery, and two an atrial septal defect. We performed fistula closure either by ligating or transsecting the fistula as well as by closure of the fistula's drainage opening. Surgery and postoperative courses were uneventful in all patients. Most of the patients (93%) were in good clinical condition (NYHA I-II) after a mean follow-up period of 6.6 years (range 3-11). Fistula closure should be performed in patients who are symptomatic or who have a hemodynamic relevant shunt. In asymptomatic patients and small left-to-right shunt, fistula closure should also be performed to prevent later complications. Surgical fistula closure should be employed in patients with larger and more complex fistulae, especially if interventional therapy failed, and for patients with additional cardiac conditions that necessitate surgery.