Objective: To explore the clinicopathologic features of the kidney carcinoma associated with Xp11.2 translocation/ translocation, transcription factor binding to IGHM enhancer 3 (TFE3) gene fusion. Methods: The clinical data, histologic findings and immunophenotype of a new case of the kidney carcinoma associated with Xp11.2 translocation/TFE3 gene fusion were reviewed, and the related literature was also reviewed. Results: A 16-year-old Chinese boy presented with right flank pain and gross hematuria for 4 days. Grossly, The tumor ( 3.0 cm × 2.5 cm× 2.0 cm) was soft and the cut surface was brown. Microscopically, the tumor predominantly composed of delicate papillary lined by well defined tumor cells. Tumor cells are clear and rich in cytoplasm, displaying red eosinophilic particleswith vesicular chromatin, obvious nucleoli and rare mitoses. Immunohistochemically, the renal cell carcinoma (RCC) and TFE3 were positive; the cytokeratin (CK), epithelial membrane antigen (EMA), and vimentin were focally positive; but the cluster of differentiation 34 (CD34), melanoma marker antibody (HMB-45) and desmin were negative. Conclusion: The kidney carcinoma associated with Xp11.2 translocation/TFE3 gene fusion is rare, which mainly occurs in children and young adults. Immunohistochemstry, fluorescence in situ hybridization and karyotype analysis are useful methods for this cancer diagnosis or differential diagnosis. The nephrectomy is often adopted for treatment, and the prognosis is hard to predict. The most frequently clinical symptoms are hematuria.