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Fibrosarcoma is a malignant mesenchymal neoplasm of fibroblasts that rarely affects the thyroid gland and can cause local re-currences or metastasis. We describe a case of primary fibrosarcoma of the thyroid in a 31-year-old man. Microscopically, the tumor showed highly cellular proliferation of spindle cells with cytologic atypia and mitotic figures. Immunohistochemically, the cells only showed immunoreactivity for vimentin and negativity for S-100 protein, CK, CD31, CD34, TTF-1, desmin. Based on clinical, radiological, histological and immunohistochemical findings, the final diagnosis was intra-thyroid fibrosarcoma. The treatment of choice is radical surgery. Radiation therapy is used as adjuvant treatment. So, we review the clinical, radiologic, histologic findings and treatment modalities of (Korean J Otolaryngol 2007 ;50 :272-4)