Immunohistochemistry (IHC) showed tumor cells diffusely expressed TFE3, and fluorescence <i>in situ</i> hybridization (FISH) demonstrated disruption of the TFE3 locus, confirming the diagnosis of Xp11.2 tRCC, the most common subtype of MiTF tRCC. Here, TFE3 is linked to renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.