Combined heart-lung transplantation with cyclosporine is reported in a 26-year-old patient who presented with end-stage pulmonary lymphangioleiomyomatosis. The operation was successful and the patient's rehabilitation excellent over the first 7 postoperative months. She then developed obliterative bronchiolitis of unknown origin. To our knowledge, this is the first published report of an out-hospital survival after heart-lung transplantation for terminal nonvascular lung disease.