Although legislation regarding organ transplantation from brain-dead donors in Japan was approved in 1997, no heart-lung transplantation procedures were performed for some time. Herein, we report the first case of successful heart-lung transplantation in Japan. The recipient was a 35-year-old man with Eisenmenger syndrome, caused by a double outlet right ventricle, who had been suffering hemoptysis and continued NYHA class 3-4 symptoms for 66 months since being registered on the waiting list. He underwent heart-lung transplantation from a 30-year-old donor. The organ ischemic time was 247 min, the postoperative course was uneventful, and the patient was discharged 66 days after the transplant surgery.