Introduction/Background Epithelioid sarcoma (ES) is rare malignant soft tissue tumor. Proximal-type epithelioid sarcoma (PES) of the vulva is a rare condition.Vulvar sarcoma occurs more often in young women (mean age, 38 years).We are reporting one case:A 22 G1L1 years old Asian woman with a mobile solid mass in the upper part of the right labia major. A mass without tenderness or any superficial laceration.

Methodology The patient was scheduled for surgery. The mass was reseted with wide loal exicion and pathology reported study showed vulvar epithelial saroma .seond surgery was done for resetion of tumor. Margin was negative for tumor residue. Metastatic work up was negative and patient don’t recieved adjuant treatment

Results Physical examination was done every 3 month and she had no pathologic sign. The visit interval can be increased to six months and the follow-up can be continued for five years. She has been disease free for 60 months.She had normal vaginal delivery two years after this surgery

Conclusion Optimal treatment for PES of the vulva has not been established due to its rarity. There is no universally accepted treatment for vulvar epithelioid sarcoma . However, the initial treatment is wide surgical excision with an adequate margin (≥2 cm) . The role of adjuvant therapy also remain unclear due to the rarity of this disease. Adjuvant radiotherapy is advocated in high-grade tumors or cases with inadequate surgical margins and the high incidence of local recurrence and distant metastasis .However, the results of radiotherapy are controversial and show no statistically significant reduction in mortality . The role of chemotherapy in the adjuvant setting appears marginally effective at best for the treatment of metastatic disease

Disclosures It is important to consider vulvar sarcomas in the clinical differential diagnosis of non-specific vulvar solid lesions, in order to establish an early accurate diagnosis and appropriate treatment