Objective To investigate the clinical value of enhanced CT in the differential diagnosis of renal hyaluronsarcoma (CCSK) and nephroblastoma (WT) in children. Methods 60 suspected CCSK/WT children were admitted in our hospital. All the children were examined by enhanced CT and pathology. The results of the two tests were compared and the signs of enhanced CT were analyzed. Results 23 cases (38.33%) were diagnosed as CCSK by immunohistochemical staining of pathological sections, all of them had unilateral disease, 17 cases were on the left side and 6 cases were on the right side, the maximum diameter of the tumor was 5.26～15.62cm, and no metastatic lesions were found. There were 37 cases (61.67%) of WT, all of them had unilateral disease, 21 cases on the left side and 16 cases on the right side, the maximum diameter of the tumor was 7.14～20.14cm, and no metastatic lesions were found. According to the results of enhanced CT, five important signs of diagnosis were summarized.Among them, calcification, intratumoral hemorrhage, necrosis or cystic degeneration, and small cyst at the edge of the mass showed no difference between CCSK and WT (P>0.05). The proportion of upper CCSK was higher than that of WT (P<0.05). Conclusion Enhanced CT has significant clinical value in the diagnosis and differentiation of CCSK and WT in children, which is helpful for the diagnosis and early treatment of the disease.