Ewing sarcoma is a rare highly malignant neoplasm that is mostly encountered as a primary bone tumor of childhood. Extra osseous occurrence is less frequent and cases involving the vulva are very rare with only twelve reported to date. We report the case of a Ewing sarcoma that presented as a vulvar mass in a 28-year-old Lebanese lady and briefly review the differential diagnosis as well as the published literature regarding the clinical presentation and prognosis.