Operation for pulmonary atresia and ventricular septal defect is challenging, as in most patients the pulmonary arteries are hypoplastic, nonconfluent, or in part absent. In these cases, combined heart-lung transplantation may be the surgical treatment of choice. In the described case, the morphology of pulmonary atresia and ventricular septal defect was present in combination with completely atretic main, left, and right pulmonary arteries. In this patient, successful heart-lung transplantation was performed.