An anomalous origin of the left coronary artery from the pulmonary artery is a very rare congenital malformation. We examined the early and late results of 9 patients (4 males and 5 females, mean age at operation: 6.5 years +/- 6 years). Eleven operations were performed in these 9 patients. The following types of operation were performed: ligation of the left coronary artery close to the pulmonary artery in one case, coronary artery bypass grafting in 3 patients, reimplantation of the LCA into the aorta in 3 patients and interpulmonary artery tunnel repair ("tunnel operation") in 4 instances. These operations were combined with plastic reconstruction of the mitral valve (1 patient) or aneurysmectomy of the left ventricle (3 patients). There was no intra- or early operative mortality. After a mean follow-up of 7.3 years, all patients are alive and in New York Heart Association functional class I, except one patient suffering from dyspnoea during moderate exercise. In two patients complications requiring reoperations occurred after 4 and 100 months respectively: stenosis of the distal anastomosis after coronary artery bypass grafting using saphenous vein and thrombosis of the intrapulmonary tunnel. We conclude that an anomalous origin of the left coronary artery from the pulmonary artery is best operated by reimplantation of the vessel into the aorta at any age of life. Concomitant pathology may be corrected simultaneously, without increasing operative mortality.