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Background: Perivascular epithelioid cell tumors are rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. They are considered ubiquitous tumors and have been described in different organs, but gastro-intestinal PEComas are diseases of extreme rarity., Methods: We report a case of a 51-year-old woman, without a medical history of tuberous sclerosis complex, affected by abdominal PEComa, adhering tightly to the jejunal loop and to the spleen., Results: During the surgical operation, a large abdominal mass was found, and surgical resection was carried out. Definitive histologic and ultrastructural findings were consistent with PEComa., Conclusion: Given the rarity of GI-PEComas and the lack of cases reported in the literature, we want to emphasize the importance of conducting further studies in this regard, to better describe their biological behaviour., Key Words: Gastro-intestinal tumor, PEComa, Perivascular epithelioid cell tumors.