To review the nursing experience of a child with pulmonary cystic fibrosis caused by autosomal gene mutations and undergoing bilateral lung transplantation. Aiming at the characteristics of the child such as low weight and difficult perioperative management，we adopted goal-ori- ented volume management，and nurse-led cough reflex remodeling，effective posture drainage and progressive lung re- cruitment training management. Treatment and nursing of digestive tract complications，and immunosuppressive drug care were also implemented. After 24 days of hospitalization and nursing，the child was discharged smoothly.