This report describes a patient with a 15-year history of schwannoma (peripheral nerve sheath sarcoma) who developed extensive pulmonary metastases associated with hypoxemia. Treatment with chemotherapy consisting of cyclophosphamide, vincristine, Adriamycin, and imidazole carboxamide resulted in a complete remission lasting 17+ months. Malignant schwannoma should probably be regarded as a drug sensitive neoplasm.