BACKGROUND: Although initial presentation of sarcomas in the head and neck region is relatively common, involvements of the larynx by rhabdomyosarcoma either primary or metastatic have been reported rarely. CASE REPORT: A case of rhabdomyosarcoma in the right thigh, which involved laryngeal structures three years after the diagnosis, is presented. CONCLUSIONS: The laryngeal involvement by primary and metastatic rhabdosarcomas is very rare, however when it occurs it can result in life treating upper airway obstruction. After emergency intervention, radiotherapy and / or chemotherapy must be undertaken for long-term benefit and surgery may be helpful only in selected cases.