The association of biliary atresia (BA) with congenital heart diseases has been extensively described, and there are a number of reports on the outcomes of patients in this group who undergo liver transplantation (LT). The intraoperative management and the timing of LT for patients with end-stage liver disease are matters of debate, especially when complex heart diseases are involved. This report describes the outcome after LT for a pediatric recipient with BA and hypoplastic left heart syndrome. The patient underwent Norwood-Sano and Glenn procedures for heart palliation before LT. He was cyanotic, was severely malnourished, and had complications secondary to chronic liver failure. At the time of transplantation, the child was 16 months old and weighed 5175 g. Despite the critical clinical scenario and the long hospitalization period, there were no cardiac, vascular, or biliary complications after LT. At the age of 48 months, the patient was awaiting the final cardiac repair. In conclusion, the presence of complex cardiac malformations may not be a contraindication to LT. An experienced surgical team and a multidisciplinary approach are key to a successful outcome. Liver Transpl 19:322–327, 2013. © 2013 AASLD.