Breast angiosarcoma (BA) is a rare and highly aggressive malignancy with a poor prognosis. It can occur spontaneously or be associated with factors such as radiation therapy or chronic lymphedema. The etiology and pathogenesis are unclear, and clinical symptoms and imaging findings lack specificity, making diagnosis challenging. There is no clear guideline for surgical treatment, but the primary goal is surgical excision with optimal margins. The choice of surgical approach should consider tumor size, pathological type, and patient preferences. Postoperative adjuvant therapies, including radiotherapy and chemotherapy, are not standardized, and their efficacy is uncertain. The prognosis for BA is poor, with 5-year survival rates ranging from 28% to 54%. Advanced age, tumor size, and histological grading are significant prognostic factors. Accurate diagnosis requires thorough histopathological sampling and immunohistochemical analysis. Standardized treatment guidelines are lacking, and a multidisciplinary approach involving breast surgeons, medical oncologists, radiologists, and plastic surgeons is recommended to improve patient outcomes.Breast angiosarcoma (BA) is a rare and highly aggressive malignancy with a poor prognosis. It can occur spontaneously or be associated with factors such as radiation therapy or chronic lymphedema. The etiology and pathogenesis are unclear, and clinical symptoms and imaging findings lack specificity, making diagnosis challenging. There is no clear guideline for surgical treatment, but the primary goal is surgical excision with optimal margins. The choice of surgical approach should consider tumor size, pathological type, and patient preferences. Postoperative adjuvant therapies, including radiotherapy and chemotherapy, are not standardized, and their efficacy is uncertain. The prognosis for BA is poor, with 5-year survival rates ranging from 28% to 54%. Advanced age, tumor size, and histological grading are significant prognostic factors. Accurate diagnosis requires thorough histopathological sampling and immunohistochemical analysis. Standardized treatment guidelines are lacking, and a multidisciplinary approach involving breast surgeons, medical oncologists, radiologists, and plastic surgeons is recommended to improve patient outcomes.