Abstract: To analyze the clinical features of severe air leakage syndrome in neonates and improve the clinicians understanding of the disease.A retrospective analysis of the clinical data and imaging features of a severe gas leak syndrome in the Department of Neonatology of the Second Affiliated Hospital of Xi'an Medical University in December 2016.Combining the reports of the severe gas leakage syndrome in neonates.The Chinese keywords“neonatal gas leakage, neonatal gas leakage syndrome”and“air leak syndrome, in the neonatal”are used as English keywords in CNKI and Pubmed databases Searched for.The search scope was from January 2009 to December 2017.Screening reports on cases of severe air leakage syndrome, detailed record of relevant information, and review of the literature, we summarized the causes, clinical features, and diagnosis and treatment methods of the disease.A total of 16 Chinese articles were searched.Among them, 13 articles reported the analysis of the causes of air leakage and air leakage treatmented by high-frequency ventilation.13 articles only mentioned severe air leakage, they were not described and studied in detail and were excluded.Three cases reported severe air leakage syndrome.The number of cases was 5 cases.A total of 12 foreign language literatures were retrieved, 4 case reports were reported, and the number of cases was 4 cases.In addition, there were 10 cases of this case, of which 6 cases were dead, 3 cases survived, and 1 case was not reported.Severe air leakage syndrome has a rapid progression and high mortality.It should be prevented and treated early.