Purpose To investigate the clinical pathological features, pathological diagnosis and differential diagnosis of the non-Wilms’ renal tumors in children. Methods The clinical pathological data and immunohistochemical findings of 15 cases of non-Wilms’ renal tumors were retrospectively analyzed with review of the relevant literature. Results In 7/15 (46. 67%) cases of cystic nephroma ( CN) , microscopically the variable size of cysts were lined by a single layer flattened, cuboidal epithelia, separated by fi-broblastic stroma;in 4/15 (26. 67%) cases of rhabdoid tumor of kidney (RTK), histological characteristics were rhabdoid cells and cytoplasmic inclusions;in 2/15 (13. 33%) cases of clear cell sarcoma (CCSK), the classical pattern of histology demonstrated nests, cords of ovoid epithelioid or spindled clear cells separated by fibrovascular septa, which had a marked ’ chicken wire’ pattern of small blood vessels;in 1/15 (6. 67%) case of congenital mesoblastic nephroma (CMN), the tumor was mainly composed of spindle cells arranged in fascicular pattern, in which fetal renal tissue was seen;in 1/15 (6. 67%) case of granularity renal cell carcinoma, micro-scopically, the cancer cells with eosinophilic granular cytoplasm arranged in nests, cords and tubular pattern. Conclusions Although non-Wilms’ renal tumors is a small part of all kidney tumor, it is difficult to diagnose and distinguish from other tumors before opera-tion. The pathological diagnosis is the most reliable method for differential diagnosis of non-Wilms’ renal tumor.