Although PRCC-TFE3 RCC is the first reported Xp11.2 tRCC, because of less clinicopathologic data, distinctive histopathologic characterization, recommended diagnostic criteria, and standardized therapeutic strategy of PRCC-TFE3 RCC has yet to be established. The gene discussed is PRCC; the disease is renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.