A case of an extremely unusual tumor of the larynx, pleomorphic rhabdo
myosarcoma, is presented with a review of literature. This is the fift
h case of this malignancy described in the larynx in the English langu
age literature. A histopathological diagnosis was made with immunohist
ochemistry and electron microscopy. In contrast to other reported case
s, the tumor in the present case had a very aggressive behavior. Despi
te radical surgery involving total laryngectomy and neck dissection fo
llowed by radiation therapy, the patient died of disease 8 months foll
owing treatment.