Rhabdomyosarcoma is a very uncommon highly aggressive malignant tumor originated from mesenchymal cell. It is the most common soft tissue sarcoma in children. According to histopathological classification of intergroup rhabdomyosarcoma study, there are four principal histologic varieties of rhabdomyosarcoma-embryonal, botryoid, alveolar and pleomorphic. Head and neck is most common site of the embryonal rhabdomyosarcoma, but primary site of middle ear and mastoid is rare. Authors experiences a case of embryonal rhabdomyosarcoma in middle ear and mastoid extending to brain in a 7 year old male who was admitted due to headache, otorrhea, otalgia and facial nerve palsy. This case was managed with combination therapy of cheotherapy and irradiation.