Knowledge of vulvar rhabdomyosarcoma (RMS) is based on case reports and collective reviews since the pathology is extremely rare and experience of individual institutions limited. We present a case of a 15-year-old girl with large-sized vulvar embryonal RMS. The patient was treated with wide local excision of the tumor, multiagent chemotherapy and radiotherapy, achieved complete remission but 6 months after diagnosis died of toxicity. The management is discussed. Necessity of early anticipation of malignancy in girls is pointed out as it may determine outcome of therapy.