Abstract: 　Objective　To explore the potentially relevant copy number variations (CNVs) in congenital cystic lung diseases (CCLD). Methods　Clinical data of 16 patients diagnosed with CCLD and CNVs results were retrospectively analyzed. Results　Of 16 cases, 12 were males and 4 were females aged between 2 months and 12 years and 6 months. Of 16 cases, 10 cases were bronchogenic cyst, 4 cases were pulmonary sequestration, 2 cases were congenital cystic adenomatoid malformation, 1 case was congenital lobar emphysema and one case was not classified. These cases presented mainly with fever, cough, and sputum without specificity. Six CNVs with unknown clinical significance were found in two patients. Abnormal amplification of HDAC8 gene was found in 4 patients diagnosed with BC. Conclusions　CCLD is less likely to be associated with the CNVs; HDAC8 gene may be related to bronchogenic cyst.