TFE3-rearranged renal cell carcinoma, as the name indicates, harbors TFE3 gene translocation which fuses with one of several other genes, such as ASPL (ASPSCR1), PRCC, SFPQ, CLTC, PARP14, RBM10, NONO, and MED15 [3–11]. Here, TFE3 is linked to hereditary clear cell renal cell carcinoma.