Sarcoma of the thyroid gland is a very uncommon malignant tumor (<1% of thyroid cancers) characterized by severe local course and rapid metastatic dissemination and very poor prognosis. We report the case of an 84-year-old woman hospitalized for expiratory dyspnea, severe malnutrition and swallowing disorders leading to bronchial infection. She had a voluminous (20 cm) extra-thoracic right-neck goiter with a considerable superficial venous pattern that had developed on an old goiter before growing recently. CEA and calcitonin levels were normal. The cervical-thoracic CT demonstrated a partially necrosed heterogeneously hypodense tumor compressing the esophagus and the trachea with displacement of adjacent structures which were not directly invaded. Large areas of necrosis and a probable metastatic image measuring 3 cm in the right median pulmonary lobe were observed. Macrobiopsy disclosed grade 3 sarcoma. Complete resection delivered a 3.170 kg tumor. Histology confirmed the diagnosis of sarcoma without neoplastic extension. Apart from right recurrent palsy, the initial post-operative period was satisfactory and the patient was discharged. Four and a half months later she was rehospitalized with local recurrence with a large metastatis in the right lung. She died two weeks later.