Pathological examination revealed that the case was considered to be XP11.2 tanslocations/TFE3 gene fusions associated renal cell carcinoma with a size of 9× 8 × 8 cm, vascular region showed invasion of carcinoma, and left renal hilar lymph nodes were with metastases (2/2). The gene discussed is TFE3; the disease is renal cell carcinoma.