Objective Analyse and report the diagnosis and treatment process of vulvar epithelioid sarcoma, it provides a basis for rare case in clinic. [Methods] Through the analysis of the patient's il ness, treatment, and pathology examination, and the effect of the treatment of prognosis of vulvar epithelioid sarcoma of the summary, truly record the occurrence of vulvar epithelioid sarcoma development, treatment and prognosis. [Results] The intraoperative pathologic examination in patients diagnosed the vulvar epithelioid sarcoma, and it used the removal of expanding. Now the patient is not recurred. [Conclusion] Vulvar epithelioid sarcoma is clinical y rare, needs combined with immunohistochemical examination at the time of diagnosis, removal of local expansion is the effective method to treat, it is not exactly curative effect of chemotherapy and radiotherapy.