Objective: To investigate the clinical pathological features, classification, differential diagnosis and treatment of Pediatric renal cell carcinoma. Methods: To valid our proposition, we reclassify children kidney carcinoma under both light microscope and immune histochemical detection. The samples we used were consisted of two male cases and one female case, whose age ranged from 5.5 to 9 years old. All of the three samples were collected from children's hospital of hunan province since 2003. Results: In our experiment, 1 case with papillary structures arranged in translucent cytoplasm of cancer cells mainly, meanwhile fibers, blood vessels and inflammatory cells infiltrated between nipple accompanied by more calcified bodies; The other two were solid nests or duct-like arrangement of the eosinophilic granular cancer cells based in, regional distribution of a small amount of focal atypical papillary structures, but no calcified bodies in visible. Immunohistochemistry results: The expressions of case 1 were TFE3, Vimentin, CK-pan and CEA; case 2 expressed Vimentin, CK-pan, CEA and p53; case 3 expressed Vimentin, CK- pan, CEA, NSE, CgA, Syn and Ki-67. Conclusion: Renal cell carcinoma rarely happened to children, The HE form in clear cell types arranged in papillary structure should be combined with the TFE3 protein immunohistochemical and gene detection methods in diagnosis. Preoperative intravenous chemotherapy can improve the rate of complete tumor resection. The overall prognosis in children is better than adult, however renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions is poorer than clear cell renal cell carcinoma as to prognosis. In addition, since its performance is inert in childhood, better prognosis often requires long term observation.