Objective   To investigate the clinical efficacy and prognosis of lung transplantation in the treatment of cystic fibrosis (CF).

  Methods   Clinical data of one patient with end-stage CF undergoing allogeneic bilateral lung transplantation were retrospectively analyzed. Clinical characteristics, diagnostic methods and treatment strategies of the CF recipient were summarized.

  Results   The recipient had suffered from relevant symptoms since childhood including repeated cough and purulent sputum for 30 years, complicated with recurrent pulmonary infection combined with acute exacerbation, chronic sinusitis and extremely severe malnutrition. Prior to lung transplantation, the patient had to depend upon the invasive ventilator due to respiratory muscle weakness, and admitted to intensive care unit (ICU) for a long time. Imaging examination revealed multiple cystic columnar bronchiectasis accompanied with infection in bilateral lungs. The diagnosis of CF was further confirmed by sweat test and gene detection. The recipient underwent bilateral lung transplantation on August 17, 2017 and received rehabilitation treatment. The lung function was gradually restored to normal. The recipient had obtained the same quality of life to the healthy counterparts since the date of manuscript submission (over 2 years).

  Conclusions   Lung transplantation is an efficacious treatment for end-stage CF, which can not only save patients' lives, but also significantly improve the quality of life of patients.