Objective：To investigate the clinicopathological feature，diagnosis，differential diagnosis，treatment，prognosis and misdiag－nosis causes of uterine perivascular epithelioid cell tumor（PEComa），so as to reduce misdiagnosis and mistreatment. Methods：Misdi－agnosis data of one patient with PEComa admitted to our hospital were retrospectively analyzed，and relevant literatures were re－viewed. Results：A middle-aged female patient was admitted to the hospital due to “39+5 weeks of menopause，without abdominal pain，requiring hospitalization and delivery”. During pregnancy，ultrasound examination indicated：intrauterine pregnancy and uterine fi－broids. Cesarean section + laparoscopic myomectomy were performed in our hospital. Postoperative pathological examination sug－gested PEComa. Postoperative follow-up lasted for one year and the patient is now surviving without tumor. Conclusion：Uterus PEComa is a relatively rare disease，which is easily misdiagnosed as uterine leiomyoma due to its lack of imaging and clinical characteristics. The final diagnosis requires pathological examination and immunohistochemistry. At present，surgical treatment is an effective method；efficacy of chemoradiotherapy has not been determined. Long-term follow-up is required after surgery.