End-stage lung disease in Cystic Fibrosis (CF) now is considered to be one of the indications for heart-lung or double lung transplantation. Results of this surgery for 50 or so CF patients in the US and Europe are about the same as for other diseases, although there are some postoperative problems specific for this diagnosis. These include: need for higher oral dosages of cyclosporine, likelihood of precipitation of diabetes mellitus with high dosage corticosteroid therapy for acute lung rejection, constant threat of pathogens remaining in the sinuses, increased likelihood of drug toxicity to the liver and kidneys, and need to make a psychological transition from a patient with a fatal disease to one with optimism about the future. Although improved postoperative management likely will improve postoperative mortality and morbidity, scarcity of donor organs and the high cost of the procedure will limit the impact of this procedure on the general CF population.