Rhabdomyosarcoma is a very uncommon highly aggressive malignant tumor originating from mesenchymal cells. It is the most common soft tissue sarcoma in children. Head and neck is the most common site but larynx is very uncommon as a primary site. Rhabdomyosarcoma has a poor prognosis, but recently combination treatment of surgery, radiation, and chemotherapy improved the prognosis. Recently the authors have experienced a case of rhabdmyosarcoma of the larynx in 7 year old boy. So the authors report this case with review of literatures.