Rhabdomyosarcoma is a diagnostic and therapeutic problem in the management of pediatric tumor, representing the most common soft tissue sarcoma of childhood and rhabdomyosarcoma of ear region has been considered uniformly lethal. According to histopathological classification of intergroup rhabdomyosarcoma study, rhabdomyosarcoma is subtyped into pleomorphic, alveolar, embryonal and botryoid type. The embryonal rhabdomyosarcoma arises from unsegmented and undifferentiated mesoderm and is more common in head and neck region(particularily orbit, bit, nasopharynx and middle ear), retroperitoneum, bile duct and urogenital tract. The prognosis has improved markedly in recent years by combination of surgery, radiation therapy and multidrug chemotheraphy after first report of intergroup rhabdomyosarcoma study in 1977. Authors experienced a case of embryonal rhabdomyosarcoma in aural region in a 5 year old female who was admitted due to otorrhea and otalgia. This case was managed with combination therapy of surgery, irradiation and chemotherapy(Vincristine, Actinomycin-D and Cyclophosphamide).