Lung transplantation should be considered in cystic fibrosis patients with end-stage lung disease. The optimal time for pretransplantation evaluation rely on spirometric and gazometric criteria, as well as on the decline of pulmonary function and the degree of quality of life's impairment. Non-pulmonary complications of cystic fibrosis such as diabetes, osteoporosis and poor nutritional status should be evaluated, prevented and treated. Post-transplantation survival of cystic fibrosis patients is not different from that of other groups of pulmonary transplanted subjects, and is surprisingly not affected by chronic bacterial and fungal colonization of the airways.