The case of a 22-year old male patient who presented with a congenital coronary arterial fistula from the right coronary artery to the right ventricle is described. The arterial fistula had led to an aneurysm of the right sinus of Valsalva and the proximal right coronary artery. The aneurysm was incised from the aorta and the fistula closed with a pericardial patch. The diameter of the aneurysm was reduced by plication. Successful occlusion of the fistula and the competence of the aortic valve were confirmed by transoesophageal echocardiography.