Approximately 40 cases of PEComas of the urinary bladder and 4 cases of PEComas of the prostate have been reported in the literature; only 9 cases of bladder PEComas and 1 case of prostatic PEComas with TFE3 rearrangement have been described in the literature [3–5]. The gene discussed is TFE3; the disease is neoplasm with perivascular epithelioid cell differentiation.