Rhabdomyosarcoma is not a condition common in the general population and especially the rhabdomyosarcoma of the ear is a condition which is rare. Rhabdomyosarcoma is one of the most common malignant mesenchymal neoplasms of childhood. Embryonal type is most common and is characterized by a rapid growing and poor prognosis. Common clinical features are out of proportion to the symptoms. Constant awareness of this condition should be maintained, and atypical symptoms or finding should be thoroughly investigated. If survival is expected, immediate and energetic treatment must be carried out. Combination therapy (surgery, irradiation and chemotherapy) of embryonal rhabdomyosarcomas of the head and neck in children is recommended. The authors reporting a rare case of rhabdomyosarcoma involving right ear and mastoid in a 3-year-old boy.