Purpose To investigate the histopathological features of cystic lung diseases ( CLD) , and to discuss the timing of clinical interventions. Methods HE and immunohistochemical staining were performed and reviewed in 125 cases of CLD. Results 125 ca-ses of CLD aged from birth to 11 years and 6 month, with an average age of 23. 0 months, median age 15 months, of which 60 cases were less than 1 year (48. 0%). 75 cases were male and 50 cases female, with male to female ratio of 1. 5 ∶ 1. Grossly, 50 cases showed single or multiple cysts with the size 0. 5 ~8. 0 cm in diameter, which did not communicate with bronchial cavity. 18 cases showed honeycomb cysts with the diameter of 0. 1~2. 0 cm. 26 cases were solid lesions without visible cysts. 21 cases were observed lung abscess with thick and rough wall and pus inside. 7 cases of emphysema showed microcysts with crepitation. 2 cases were identi-fied cystic and solid masses, with fish-fresh like cut surface. Histopathologically, 94 cases (75. 2%) were related to congenital bron-chopulmonary dysplasia in 125 cases of CLD, in which there were 59 patients (47. 2%) of congenial pulmonary airway malformation (CPAM), including 29 cases of type 1 (49. 2%), 18 cases of type 2 (30. 5%), and 12 cases of type 4 (20. 3%), there were 26 ca-ses (20. 8%) of pulmonary sequestration, including 15 cases of intralobar type (57. 7%) and 11 of extralobar cases (42. 3%), 5 ca-ses were complicated with CPAM type 2, 8 cases were bronchial cyst (6. 4%) and 1 case of enteric cyst (0. 8%). Acquired lesions were detected in 31 cases (24. 8%), including 21 cases of infected lung abscess, 1 case of fungal abscess. 7 cases of emphysema, and 3 cases of pleuralpulmonary blastoma (typeⅠ1 case and typeⅡ2 cases). Conclusion Pediatric CLD is characterized as com-plexed categories. The prognosis depends on correct pathological diagnosis, combined with imaging evaluation and appropriate timing of surgery.