Most malignant schwannoma are deeply situated, and some may arise from superficial neurofibromas. 1 Solitary tumors are more prone to recognition than those associated with neurofibromatosis because the histologic appearance resembles that of other sarcomas and sometimes undifferentiated carcinomas. 2 Electron microscopy or immunohistochemical studies are needed to confirm the diagnosis of malignant schwannomas. 3 The presentation and management of an extremely rare case of malignant schwannoma in the thyroid gland is reported.