Rhabdomyosarcoma is a malignant tumor of mesenchymal origin. It is the most common soft tissue sarcoma of childhood and approximately 250 new cases are diagnosed in the U.S. each year. The most common sites for rhabdomyosarcoma are the head and neck (parameningeal, orbit, paryngeal etc.), the extremities, and the genitourinary tract. Histologically, it can be classified into embryonal, alveolar, pleomorphic, and undifferentiated. Alveolar subtype accounts for approximately 25%. Alveolar rhabdomyosarcoma is very rare and its prognosis is very poor. Especially, cases that reported from vulva are extremely rare, and informations regarding treatment and prognosis are not standardized. We present a case of a girl with alveolar rhabdomyosarcoma of vulva with a review of a literature.