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. The gene discussed is ASPSCR1; the disease is renal cell carcinoma.