A case of a 31-year-old woman with epithelioid sarcoma of the vulva which metastasized to the regional lymph node 8 years after onset of the disease is reported here. The patient first noticed a painless subcutaneous mass of 5 mm in diameter in the right labium majus at age of 21. This was excised locally at age 23, but recurred 17 months later. Although local excision was again performed, the tumor recurred and continued to enlarge very slowly. At this stage, based on the pathology of both the initial and second tumors, the diagnosis was of a benign inflammatory process. However, local recurrence and inguinal lymph node swelling occurred at age 29, and biopsy was taken. The pathology report indicated benign granulomatous changes. The slides were reconsidered and re-interpreted as epithelioid sarcoma, whereupon radical vulvectomy was performed at age 31. Vulvar epithelioid sarcoma with inguinal lymph node metastasis was first diagnosed at that time. Epithelioid sarcoma of the vulva is an exceedingly rare tumor, and only 15 cases have been reported thus far in the literature. Early diagnosis and curative treatment of this tumor may be problematic for gynecologists because of its rarity and therefore little-known characteristic clinical behavior and histology. Radical vulvectomy or extensive local excision with inguinal lymphadenectomy at the time of diagnosis is recommended as the treatment of choice.