Abstract:【Abstract】 Objective：To explore the etiology and clinical features of Multiple cystic lung disease (MCLD) in children. Methods：Retrospective analysis of the clinical data of 25 cases of MCLD in Children's Hospital Affiliated to Chongqing University of Medical Sciences from March 2004 to December 2012. Results：(1) The causes of MCLD in children were: 12 cases (48%) with Bronchopulmonary dysplasia (BPD), 7 cases (28%) with Langerhans cell histiocytosis (LCH), 3 cases (12%) with idiopathic pulmonary fibrosis, and 3 case of unknown reason. (2) Clinical manifestations of respiratory system: 22 cases (88%) had tachypnea, 16 cases (64%) had cough , 15 cases (60%) had dyspnea, Extrapulmonary manifestations were various and specific: ear pus overflow in 3 cases (12%), head mass in 2 cases (8%), 6 patients with anemia (24%),lymph nodes in 5 cases (20%), 7 (28%) cases with hepatosplenomegaly, 3 cases of skin rash (12%), clubbing in 1 cases(4%). (3) 25 cases were had Computed tomography(CT) examination, and were all showed MCLD . (4) 3 cases of IPF had pulmonary function tests, the results suggestive restrictive ventilatory defect. Conclusion：(1) The most causes for children with MCLD in this study were BPD, LCH, IPF. (2) The common clinical manifestations of MCLD in children was respiratory system, and were unspecific, but extra-pulmonary manifestations were various and specific, they were important value in diagnosis of MCLD. (3) The diagnosis of MCLD should combine with the history, extrapulmonary manifestations, pulmonary function tests and chest CT. (4) Histopathological examination is a diagnosis tool of some disease, if the disease could not diagnosis through the clinical manifestations and image features, the pathological biopsy should be performed.