ObjectiveTo study the clinical features,pathogenesis and differential diagnosis,and explore the imaging characteristics of congenital cystic adenomatoid malformation of the lung (CCAM) in children,in order to improve the knowledge of imaging findings of the disease. MethodsWe retrospectively analyzed the clinical data and imaging characteristics of three patients proven to have CCAM of the lung both by surgery and pathology between March 2011 and March 2013.Related literature was also reviewed,and two out of the three patients were followed up for two years. ResultsAll CCAM lesions in this study involved unilateral lower lobe,including two left lung and one right.Two patients had multiple large cysts containing gas,and one had multiple honeycomb-shaped vesicles.Histopathological examination showed that two cases were classified as Stocker type I and one as type Ⅱ,without type Ⅲ cases.Surgeries proved that the malformation was unilateral in all patients without connection with the bronchia.All patients with CCAM got complete cure by operation.The two-year postoperative follow-up showed an excellent recovery in all the patients. ConclusionImaging examination is the main basis for discovery and diagnosis of CCAM.The CT manifestations of CCAM are featured,and according to CT examination,pathological subtypes of the disease can be predicted.Being familiar with these characteristics is helpful for its diagnosis.