Although over the past 30 years, numerous fusion partners have been identified in TFE3-rearranged renal cell carcinoma and PEComa, ASPL/ASPSCR1, PRCC, SFPQ, NONO, and MED15 remain the most frequent in both groups of neoplasms. The gene discussed is ASPSCR1; the disease is neoplasm with perivascular epithelioid cell differentiation.