Lung transplantation is a valid treatment in cystic fibrosis patients at end stage of the disease. It is decided when the vital prognosis seems to be engaged within two years. Patients are put on the waiting list after they have been checked out according to their individual potential risks and difficulties. The waiting time on list may be prolonged and should be used to optimize nutritional status (gastrostomy) and to take into account the psychological aspect of the project. The choice between double lung transplantation and heart lung transplantation depends on teams experience. Survival rate is increasing (until 90% and 78% survival rate at 1 and 5 years). Contra-indications are represented by a dependence on invasive assisted ventilation before referral to transplant, and adhesions after surgical pleurectomy. Long term results depend on bronchiolitis obliterans which is for some teams an opportunity to discuss the possibility of retransplantation.