BACKGROUND Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are aggressive malignancies characterized by translocation involving the EWS gene. Primary extraskeletal ES/PNET rarely affects the female genital tract. CASE We report the case of a 20-year-old female, who presented with a rapidly growing painful mass arising from the left labium majus for six months. Multiple lesions in bones and nodules in lungs were considered metastasis. The diagnosis was established by immunohistochemical stain and reverse transcriptase polymerase chain reaction analysis. The patient ceased treatment and died of respiratory failure one month after discharge. CONCLUSION Vulvar ES/PNET mainly affects adolescent females. ES/PNET should be considered in the differential diagnosis of tumors involving the lower gynecologic tract in adolescent female.