Rhabdomyosarcoma is a relatively common solid tumor in infants, children and adolecents and highly malignant tumor originating form mesenchymal cell. This tumor occurs any portion of the body, especially head and neck, extremities and genitourinary system. Combination therapy(surgery, irradiation and chemotherapy) is recommended. We have recently experienced a case of rhabdomyosarcoma arising in the temporal bone in 8-year old boy, and obtained relatively good result after combination therapy. So we report this case with some review of literature.