Abstract: Perivascular epithelioid cell tumors（PEComas） are very rare mesenchymal neoplasms composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. They have been described in different organs and are considered as an ubiquitous tumors. Its biological behaviour can be divided into benign, malignant and uncertain malignant potential, but the criteria for diagnosis of malignancy have not been fully established due to the rarity of the tumor. The uterus is the most common anatomic site for PEComas occuring in the gynecological tract. Most cases behave in a benign fashion,and the precise etiopathogenesis of PEComas is unclear, some cases are related to the genetic alterations of tuberous sclerosis complex. For the disease is lack of specificity in clinical manifestation and imaging changes，the diagnosis relies mainly on histopathology. At present, surgical excision is thought to be the most direct and effective treatment, then followed by adjuvant radiotherapy and chemotherapy, but its efficacy is uncertain. Recently，reports on recurrence and metastasis of malignant PEComas are gradually increasing, and the prognosis is poorer. In this review，we carry out a comprehensive survey based on published data and discuss our current understanding of the clinicopathologic features，diagnosis and differential diagnosis,treatment and prognosis of PEComas occurring in the female genital system.

Key words: Perivascular epithelioid cell tumors, Genital diseases, female, Pathology, Therapy, Prognosis