We report a case of a heart transplantation in a 12-year-old girl 9 years after extensive lung resection and adjuvant chemo- and radiotherapy for intra-thoracic embryonic rhabdomyosarcoma. She had restrictive cardiomyopathy with severe heart failure and chylous ascites. She was treated successfully with orthotopic heart transplantation and her symptoms of chylous ascites subsided gradually after transplantation. Her abdominal girth decreased from 79 cm before the transplant to 53 cm 9 months after the transplant.