Introduction: Perivascular epithelioid cell tumors (PEComa) are a very uncommon mesenchymal cancer with uncertain malignant potential.
Materials and methods: A computerized research on Pub Med was performed regarding bladder PEComa. We evaluated the literature cases and described a case of bladder PEComa.
Results: PEComa of the bladder is a very rare neoplasm and only 15 cases have been reported so far.
Conclusion: Bladder PEComa involves young subjects of both genders, mostly under 40 years of age. Surgical treatment (partial or radical cystectomy) is the most common management.A long-term follow-up and larger series are required to better understand the best clinical approach to bladder PEComa.