Objective To analyze the clinical data of connective tissue disease-associated interstitial lung disease (CTD-ILD) in children, and to improve the understanding of CTD-ILD in children. Methods The clinical data of 53 hospitalized children with CTD-ILD from January 2012 to December 2021 were retrospectively analyzed, including clinical manifestations, chest HRCT, pulmonary function, treatment and outcome, etc. Results Among the 53 children with CTD-ILD, 23 children were diagnosed as ILD by HRCT at the same time of CTD diagnosis, 4 children had ILD onset, and 26 children were diagnosed as ILD with a time span of 15 days to more than 7 years. The common symptoms of respiratory system were cough (47.2%), shortness of breath (28.3%) and rales (26.4%). But there were 21 cases without respiratory symptoms, and ILD was diagnosed according to HRCT. Chest HRCT showed patchy shadows (64.2%), striped shadows (55.6%), streak shadows (37.7%), grid/honeycomb shadows (24.5%), cystic low-density shadows (24.5%) and ground glass shadows (22.6%), etc. The main pulmonary function abnormalities were diffusion dysfunction (46.5%), restrictive ventilation dysfunction (39.5%) and small airway dysfunction (32.6%). After treatment with glucocorticoids, immunosuppressants and biological agents, 48 children showed improvement in clinical symptoms. Chest HRCT showed improvement in 20 children, no significant change in 21 children, and progress in 6 children. Pulmonary function examination showed improvement in 17 children, no significant change in 11 children, progress in 3 children, and normal in 8 children. Conclusions The clinical symptoms of CTD-ILD in children are lack of specificity, and chest HRCT and pulmonary function tests are helpful for early detection of pulmonary lesions, so relevant examinations should be actively completed and regular follow-up should be conducted. Treatment options are glucocorticoids combined with immunosuppressants and/or biological agents, but the use of biological agents in children needs further study.