Abstract
Malignant peripheral nerve sheath tumors are highly aggressive tumors and they arise either de novo or from preexisting benign schwannoma or neurofibromatosis. Malignant peripheral nerve sheath tumor of the larynx is an extremely rare neoplasm, for which accurate diagnosis is difficult and the therapy is usually delayed. Although wide surgical excision is the mainstay of treatment, radiation therapy also plays an important role in the control and treatment of locally recurrent disease and non resectable cases. Here we are reporting a case of malignant peripheral nerve sheath tumor of the larynx without definite evidences of von Recklinghausen's disease or preexisting benign peripheral nerve sheath tumor.