Objective To analyze the CT findings and clinical features of congenital cystic adenomatoid malformation of the lung (CCAM) in children. Methods 29 cases of children who were diagnosed with CCAM by surgery and pathology in our hospital between June 2010 and May 2015 were enrolled in the study. All the children underwent CT examination after admission, and the clinical data were complete. The clinical manifestations were analyzed. The CT imaging findings of CCAM in children were summarized. Results Among 29 cases with CCAM, there were 24 cases with right lung involvement, including 13 cases of superior lobe, 9 cases of inferior lobe and 2 cases of inferior and middle lobe involvement detected by CT. There were 5 cases with left lung involvement, including upper lobe in 2 cases and lower lobe in 3 cases. Among them, 23 cases were large cystic type, with single or multiple thin wall air cysts. There were irregular and multiple line-like separations inside; 6 cases were follicular type. CT showed honeycomb-like changes and multiple cysts without intracystic gas-liquid free flat. There was no abnormal density shadow in lung tissues around cysts and no normal aerated lung tissues. There were presence of right shift of mediastinum or no changes. Conclusion The CT findings of children with different pathological types of CCAM are specific. The coincidence rate of CT typing and pathology is high, which can be used as an important means for screening CCAM.