Introduction: Perivascular epithelioid cell tumors (PEComas) of the bladder are infrequent localisation of this mesenchymal cancer with uncertain malignant behavior. Case report: We report the case of a 74 years old women who was diagnosed a malignant PEComa of the bladder. She necessited radical cystectomy with orthotopic ileocaecal pouch reconstruction. Histology and immunohistochemistry confirmed the diagnosis of a malignant PEComa of the bladder. We evaluate the literature cases to adjust the prognosis criteria. Conclusion: Evolution and prognosis evaluation remain hard and could necessitate a radical surgery. Prognosis criteria for the bladder PEComas have to be clarified. Keywords: Bladder, PEComas, Perivascular epithelioid cell sarcoma, Malignant, Cystectomy
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