We determined the efficacy of combined helical tomotherapy (HT) and chemotherapy in primary/recurrent unresectable rhabdomyosarcoma (RMS) of temporal bone. For this purpose, 9 patients (7 males/2 females), aged 4-9 (average: 6.89) years, with unresectable embryonal RMS of the temporal bone were treated at our hospital. The tumors had either invaded the carotid artery in the cavernous sinus (7/9) or both the cavernous sinus and the skull base foramen (2/9); 7 patients had primary and 2 had recurrent RMS. All patients underwent 2 cycles of induction chemotherapy with VIE (vincristine, ifosfamide, and etoposide), followed by concurrent HT (50-70 Gy) and chemotherapy with VE (vincristine and etoposide for 2 cycles), and 11 cycles of adjuvant chemotherapy with VIE. As a result, all patients achieved complete response, and the 2-year tumor-free survival rate was 100 %. During a follow-up of 3-51 months, all 9 patients were alive. We, therefore, conclude that the induction chemotherapy, adjuvant chemotherapy with VIE and concurrent HT and chemotherapy with VE regimen is effective in treating unresectable embryonal RMS of the temporal bone. The combined modality treatment may achieve the best chance of cure for these patients, thereby changing the therapeutic strategy from palliative to possibly curative.