Objective　To explore the clinical characteristics of congenital pulmonary airway malformation (CPAM) in children. Methods　The clinical data of CPAM in 20 children from January 2016 to December 2018 were analyzed retrospectively. Results　In the 20 children (12 males; 8 females) aged 30.4±7.6 months in average, the course of the disease ranged from 5 days to 3 years. Seventeen patients presented with recurrent cough, wheezing and fever, one presented with chest distress, and two presented with dyspnea. Bilateral lesions were found in 4 cases. There were 16 cases with unilateral lesion including 7 cases of left involvement and 9 cases of right involvement. Two children were complicated with cardiac malformations, and one had the triatrial heart. High resolution CT examination of the lung revealed large cystic type in 14 patients, mediastinal displacement in all patients , and small cystic type in 6 patients. Postoperative pathological classification showed 14 cases of type I, 5 cases of type II and 1 case of type IV. All the children received surgical treatment, and the postoperative follow-up time varied from 1 to 24 months. All children recovered well. Conclusions　CPAM is rare and can be combined with other system deformities which can be found by high-resolution CT of the lung. The confirmation of diagnosis of CPAM should be based on the histopathological examination, and the prognosis is good after the operation.