In a male patient, presenting with progressive angina refractory to medical treatment, a solitary aneurysm of the left main coronary artery without fistula, a 2 cm large atrial septal defect (ASD) and a persisting left superior caval vein were diagnosed. Successful resection with subsequent in situ repair by vein grafting of the left anterior descending artery and the circumflex coronary artery system and direct closure of the ASD are described.