While the proportion of renal cell carcinoma in adults was only 1% to 1.6%.[2] According to the reports of Argani et al, Xp11.2-RCC resulted from the fusion of TFE3 gene with 1 of 5 different genes, including ASPL (17q25), PRCC (1q21), PSF (1q34), NonO (Xq12) and CLTC (17q23).[3,4] The main clinical manifestations of this type of renal cell carcinoma include hematuria and lumbago, and also frequently occur in the clear cell renal cell carcinoma patients. This evidence concerns the gene TFE3 and hereditary clear cell renal cell carcinoma.