Objective The CT features of teratoid Wilms tumor(TWT)in children were analyzed to improve its clinical diagnostic value.Methods The clinical data and CT findings of 7 children with histopathologically confirmed TWT were ret-rospectively analyzed,and the CT classification and staging were performed to summarize its characteristics.Results The male to female ratio of the 7 children with TWT was 5∶2,and the age ranged from 6 months to 5 years old.There were 3 cases on the single right kidney,3 cases on the left,and last case on the bilateral.The tumor boundary was not clear with incomplete capsule in 4 cases.5 cases were solid cystic mass,and 4 of them showed signs of"cyst"and"tumor within tumor".There was definite adipose tissue in 5 lesions,and calcification in 5 lesions.The CT classification was type Ⅰa(3 cases),type Ⅰb(2 ca-ses),type Ⅱa(2 cases),and no type Ⅱb was found.The solid components showed progressively inhomogeneous enhancement.The tumor blood vessels were abundant in 6 cases,and the residual kidney was significantly enhanced presented as"cup-shaped".4 cases had blurred perirenal fat space,2 cases had ipsilateral renal vein cancerous thrombosis,1 case had renal pel-vis and upper ureter cancerous thrombosis,2 cases had adjacent peritoneal implantation metastases,and 3 cases had regional lymph node enlargement.There was no distant metastasis.The CT staging was stage 1(1 case),stage 11(2 cases),stage Ⅲ(3 cases)and stage Ⅴ(1 case),and the match-rate was 85.7%(6/7).CT reexamination in 3 patients after preoperative chemo-therapy showed partial tumor retreat.Conclusion The CT findings of TWT in children have certain characteristics.The fat composition,signs of"cyst"and"tumor in tumor"found in the mass are helpful for the diagnosis.The CT staging has high accuracy and can guide clinical treatment to a certain extent.