Objective To summarize the diagnosis and treatment of congenital pulmonary airway malformation in children.Methods The clinical data of 68 children with congenital pulmonary airway malformations from June 2014 to June 2017 were analyzed retrospectively,including 39 males and 29 females with mean age(1.1 ±:0.5) years old(from 2 months 12 days to 15 years),7 cases of pulmonary cyst resection in 68 cases,3 cases with lung segment resection,1 cases with lingulectomy,57 cases with lobectomy.Results 68 cases were cured and discharged without death.Postoperative short-term complications included subcutaneous emphysema in 5 cases,pleural effusion in 2 cases,pneumothorax in 2 cases.All cases were followed up well between 3 months and 1 year.Conclusion The diagnosis of congenital pulmonary airway malformation in children mainly depends on imaging examination.Because it is easy to repeat infection,and can not rule out malignant transformation,early diagnosis and early treatment should be done.Surgical resection is the only way to treat the disease.