Abstract: Perivascular epithelioid cell tumor (PEComa) is a rare clinical mesenchymal tumor with diverse biological characteristics and undetermined malignant potential. It is extremely rare in the uterus and is easily misdiagnosed before surgery. Clinicians must pay enough attention. Therefore, we now review the data of a patient with uterine PEComa, and review the relevant literature to further explore its clinical manifestations, pathological features, immunophenotype, treatment and prognosis，in order to deepen the understanding of this disease and improve the ability to diagnose and treat.

Key words: Perivascular epithelioid cell tumor, Uterine neoplasms, Mesenchymoma, Therapy, Case report