Angiosarcoma is a rare, aggressive tumor arising from the endothelial cells of blood vessels that can affect internal organs, but the most common location is the skin. The article presents current data on the molecular genetic profile, diagnosis and treatment of primary and secondary angiosarcomas of the breast. A search for relevant sources was carried out in the PubMed, Cochrane Library and eLibrary systems, publications from 2012 to 2023 were examined, 29 of which were used to write this review. Angiosarcoma of the breast is a rare aggressive tumor characterized by a high grade of malignancy and a poor prognosis. The grade of malignancy is a predictor of survival. There are no standard treatment guidelines, and the optimal approach remains surgical resection to achieve negative resection margins. The role of neoadjuvant, adjuvant chemotherapy and radiotherapy remains controversial. © 2024, ABV-press Publishing House. All rights reserved.