This report concerns a 2-year-old boy who had situs solitus and atrioventricular concordance with double-outlet left ventricle, L-malposition of the aorta, pulmonary stenosis, tricuspid stenosis, hypoplastic right ventricle, ventricular septal defect, and secundum atrial septal defect. These lesions were correctly diagnosed preoperatively, and "correction" was achieved by closing the atrial septal defect and tricuspid valve and performing a Fontan procedure using an antibiotic-sterilized aortic homograft for the conduit. The patient initially did well postoperatively but, despite vigorous diuretic therapy, developed large bilateral pleural effusions necessitating frequent aspiration. He died unexpectedly one month after the operation. Postmortem examination showed the repair to be intact. Despite his death, we are encouraged to consider this operation in such cases in the future.