Lung transplantation has become an accepted treatment for respiratory failure due to cystic fibrosis (CF). Effective means of patient selection, surgical technique, immunosuppression, and post-transplant management permit survival as good as that of transplant patients with other diseases. The new lungs do not acquire the CF ion transport abnormalities but are subject to the usual post-transplant complications. CF problems in other organ systems persist and may be worsened by some of the immunosuppressive regimens. Prolonged survival increases the risk of age-related CF and other complications. Effective medical management requires expert knowledge of CF and lung transplantation and of how their problems interact, and good communications among the participating care teams.