We report a light and electron microscopic, immunohistochemical, clinical and prognostic study of 14 patients with epithelioid malignant schwannoma. In 8 patients the tumour involved a major nerve. The tumours were rather small in most instances, the largest diameter being less than 5 cm in 7 cases. Light microscopically, they showed highly cellular areas of epithelioid, polygonal or rounded cells characteristically forming cords and rows and arranged in nodules of varying size. Spindle cell sarcoma areas as in classical malignant schwannoma were seen in 9 cases, and neurofibromatous areas in one case. Four cases were entirely epithelioid in appearance. Electron microscopically the epithelioid tumour cells showed nuclei with mostly even contours containing one or two trabecular or reticular nucleoli, cytoplasmic projections, intra-cytoplasmic myelin-like figures, intercellular junctions and discontinuous, sometimes multilayered external lamina material. The ultrastructural findings indicate that epithelioid malignant schwannoma is a tumour of neural crest derivation having features of Schwann cell differentiation. Immunohistochemically, S-100 protein was demonstrated in 7 tumours and neuron specific enolase in 3. There was a female predominance, 9/14, and a median age of 38.5 years (range 17-74). The extremities, including the hip and shoulder regions, were the most common sites, 12/14. The tumour proved highly malignant; 9 of 14 patients were dead at the time of follow-up and a high incidence of metastasis (7 of 14) was observed.