Objective：This study aims to improve the clinicians’ levels of respiratory management and treatment of children with spinal muscular atrophy（SMA）with pulmonary infection. Methods：A retrospective analysis of the clinical data of 49 children with SMA complicated withpulmonary infection admitted from January 1，2013 to July 31，2020. Results：Among the 49 cases，35 cases were type Ⅰ，14 cases were type Ⅱ，and there was no type Ⅲ patient. Compared with type Ⅱ，SMAⅠ children with lung infection had an earlier age of onset，a longer average hospital stays（P < 0.05）. The rate of cardiac insufficiency and respiratory failure in children with SMAⅠ type was significantly higher than that of patients with SMAⅡtype（P < 0.05）. The cough rate of SMAⅡ type was significantly higher than that of SMAⅠ type patients（P < 0.05）. The detection of respiratory pathogens of the SMAⅠ type is mainly multi-drug resistant bacteria and conditional pathogens，and the SMAⅡ type is mainly infected by Mycoplasma and Streptococcus pneumoniae. The rate of invasive ventilation and the use of carbapenem antibioticsin children with SMAⅠ were significantly higher than those in the SMAⅡgroup（P＜0.05）. Conclusion：Children with SMA are prone to pulmonary infection. Actively promoting the discharge of airway secretions，appropriate respiratory support and reasonable anti-infection are crucial for the treatment of SMA complicated with lung infection.