Malignant schwannoma is an aggressive neoplasm whose cell of origin and histologic characteristics remain controversial. Despite advances in diagnostic techniques, the natural history of this tumor remains uncertain. The charts of all patients with malignant schwannoma who were treated at the UCLA Center for the Health Sciences and the University of Iowa Hospital and Clinics were retrospectively reviewed. Sixteen patients received surgery and radiotherapy in combination or as sole therapy. Irrespective of treatment modality, prognosis was poor with an overall survival of 15%. Recent advances in imaging and histologic techniques that assist in establishing the diagnosis and delineating tumor location are discussed. In addition, the literature pertaining to malignant schwannoma of the head and neck is reviewed.