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Highlights
✅ Perivascular epithelioid cell tumors (PEComas) are a group of mesenchymal neoplasms with different biological presentations from benign to malignant types. Hence, we report the first description of PEComa presenting with an acute surgical abdomen. Her definite diagnoses were made based on the histomorphology results at Imam Khomeini Hospital, Tehran University of Medical Sciences, in 2020.