Angiosarcoma is a tumor of mesenchymal origin, which is rare both among sarcomas and among malignant neoplasms (MNPs) of the head and neck. It is an aggressive tumor, since it is characterized by frequent recurrence, hematogenous metastasis, has an unfavorable prognosis. Most often, angiosarcoma is localized on the head and neck, although it can be located on any part of the skin. The causes of the tumor remain unclear. There are no harbingers of angiosarcoma. The disease can occur at any age, but is more common in older people. At the initial stages of tumor development, the skin process is characterized by edema, swelling of the soft tissues, then the focus is infiltrated, the skin color above it turns red. These manifestations may resemble other skin diseases, such as atopic dermatitis, rosacea, eczema, face eosinophilic granuloma, Jessner-Kanof lymphocytic infiltration, sarcoidosis and others, which leads to diagnostic errors and incorrect tactics of patient management. Histological and immunohistochemical studies of the lesions have the key role in the diagnosis of angiosarcoma. The treatment of angiosarcoma consists in the use of combined treatment, which, in addition to surgical excision, includes chemotherapy and radiation therapy. The article describes a clinical case of a rare malignant tumor in a 71-year-old patient — angiosarcoma in the face area — and difficulties in the diagnosis.