Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 11 1 2026 08 03 51 55 Azam Zafarbakhsh Department of Female Pelvic Floor Medicine and Surgery, Isfahan University of Medical Sciences, Isfahan, Iran. AND Department of Obstetrics and Gynecology, Isfahan University of Medical Sciences, Isfahan, Iran. Fariba Behnamfar Reproductive Sciences and Sexual Health Research Center, Isfahan University of Medical Sciences, Isfahan, Iran. Parvin Rajabi Department of Pathology, Isfahan University of Medical Sciences, Isfahan, Iran. Zahra Sheikhsoleimani Department of Obstetrics and Gynecology, Isfahan University of Medical Sciences, Isfahan, Iran. Maryam Nazemi Department of Obstetrics and Gynecology, Isfahan University of Medical Sciences, Isfahan, Iran. 2026 05 31 2026 06 07 Epithelioid sarcoma (ES) is a rare malignant soft tissue tumor, with the proximal-type variant (PES) representing an uncommon and aggressive subtype. Vulvar sarcoma predominantly affects young women, with a mean age of approximately 38 years. We report a case of a 22-year-old G1L1 Asian woman presenting with a painless, solid mass in the right labia majora, without tenderness or surface ulceration. Surgical management consisted of a right hemi-vulvectomy with a 2 cm margin from the tumor. Histopathological evaluation confirmed the diagnosis of epithelioid sarcoma of the vulva, and the surgical margins were free of tumor involvement. Postoperatively, the patient was followed every three months for the first two years and annually thereafter. Over a ten-year follow-up period, no evidence of local recurrence or metastasis was observed, and the patient experienced two successful pregnancies during this time. This case highlights the importance of considering vulvar sarcomas in the differential diagnosis of nonspecific vulvar lesions to ensure early recognition, accurate diagnosis, and appropriate management. https://crcp.tums.ac.ir/index.php/crcp/article/view/1198 https://crcp.tums.ac.ir/index.php/crcp/article/download/1198/776