Objective To observe the outcome of lung metastases in Wilms’ tumor (WT) and to explore the efficacy of different chemotherapy regimens. Methods The clinical data of WT children with lung metastases who were treated and followed up regularly from 2012 to 2020 were retrospectively analyzed to explore the difference in response to different chemotherapy regimens. Results There were 20 WT children (11 boys and 9 girls) with lung metastases, and the median age was 38.0(5.8~20.0) months. There were 17 cases of favorable histology (FH) type and 3 cases of unfavorable histology (UFH) type. Single lung metastasis was found in 13 cases and bilateral lung metastases were in 7 cases. At the initial diagnosis, 1 case was diagnosed as stageⅠ, 5 cases as stageⅡ, 3 cases as stage Ⅲ and 9 cases as stage Ⅳ. Two cases were first diagnosed in other hospitals with unknown stage. Lung metastases were found preoperatively in 12 cases and postoperatively in 8 cases. Eight cases were complicated with other distant metastases. After chemotherapy, the metastatic response was complete remission in 8 cases, partial remission in 4 cases, stable in 4 cases and progressive in 4 cases. Ordered logistic regression analysis was used for multivariate analysis. The results showed that the partial regression coefficient of CCCG-WT-2016 chemotherapy regimen was -2.05, presenting significance at the test level of 0.05 (Z=-2.03, P=0.043). It was suggested that CCCG-WT-2016 regimen was superior to NWTS-5 regimen in lung metastasis response. Conclusions The objective response in children treated with CCCG-WT-2016 regimen was significantly better than that of children treated with NWTS-5 regimen.