Primary thyroid angiosarcoma is a very rare and extremely aggressive mesenchymal malignant neoplasm showing morphological and immunophenotypic evidence of endothelial cell differentiation. Early diagnosis of this tumor along with radical thyroidectomy followed by postoperative radiotherapy and chemotherapy are essential for adequate management of the patient. Currently available data on diagnosis and treatment options of this neoplasm are limited because it is a rare disease in endocrine organs. Raising awareness regarding its diagnosis can help to optimize the treatment and to improve the survival of the patient. We present the case of a 72-year-old female patient with multiple comorbidities who addressed to the hospital with obstructive respiratory symptoms: dyspnea, wheezing and hoarseness. The investigations, both clinical and paraclinical, identified a local invasive cervical mass located mainly in the left thyroid lobe, whose immunohistochemical examination confirmed primary thyroid angiosarcoma. Although this type of neoplasm is described mainly in the Alpine regions, it can appear in lower altitude regions and this tumor needs to be differentiated from a high-grade neoplasm (anaplastic thyroid carcinoma).