We present a case of Xp11 translocation PEComa occurring in the testis harboring SFPQ⁃TFE3 rearrangement in a 57-year-old male, which, to the best of our knowledge, represents the first case of primary Xp11 translocation PEComa of the testis. The gene discussed is TFE3; the disease is neoplasm with perivascular epithelioid cell differentiation.