Breast angiosarcoma (BA) is a rare, aggressive malignancy with poor prognosis, occurring spontaneously or associated with factors like radiation therapy or chronic lymphedema. Its etiology and pathogenesis are unclear, and clinical and imaging features lack specificity, leading to potential misdiagnosis. BA is classified into primary (PBA) and secondary (SBA) types, with PBA affecting younger women and SBA older women, often linked to prior radiotherapy or Stewart-Treves syndrome. Current research is limited to case reports and small studies, resulting in limited data. BA pathology varies, with histologic grading systems proposed to assess prognosis, though results are inconsistent. Immunohistochemistry, particularly CD31 and CD34, is crucial for diagnosis. Imaging modalities like MRI and contrast-enhanced ultrasound provide better detection than X-ray. Surgery remains the primary treatment, with complete resection and negative margins as the goal. Simple mastectomy is often recommended, while axillary lymph node dissection is not typically advised. Postoperative adjuvant therapies like radiotherapy and chemotherapy are debated, with no standardized protocols. Chemotherapy regimens, including taxanes and anthracyclines, show some efficacy, while anti-angiogenic therapies are promising. Prognosis is poor, with 5-year survival rates ranging from 28% to 54%. Factors like age, tumor size, and histologic grade influence outcomes. BA is challenging to diagnose and treat, requiring a multidisciplinary approach involving surgeons, oncologists, radiologists, and plastic surgeons. Further research is needed to establish standardized treatment guidelines and improve outcomes for patients.Breast angiosarcoma (BA) is a rare, aggressive malignancy with poor prognosis, occurring spontaneously or associated with factors like radiation therapy or chronic lymphedema. Its etiology and pathogenesis are unclear, and clinical and imaging features lack specificity, leading to potential misdiagnosis. BA is classified into primary (PBA) and secondary (SBA) types, with PBA affecting younger women and SBA older women, often linked to prior radiotherapy or Stewart-Treves syndrome. Current research is limited to case reports and small studies, resulting in limited data. BA pathology varies, with histologic grading systems proposed to assess prognosis, though results are inconsistent. Immunohistochemistry, particularly CD31 and CD34, is crucial for diagnosis. Imaging modalities like MRI and contrast-enhanced ultrasound provide better detection than X-ray. Surgery remains the primary treatment, with complete resection and negative margins as the goal. Simple mastectomy is often recommended, while axillary lymph node dissection is not typically advised. Postoperative adjuvant therapies like radiotherapy and chemotherapy are debated, with no standardized protocols. Chemotherapy regimens, including taxanes and anthracyclines, show some efficacy, while anti-angiogenic therapies are promising. Prognosis is poor, with 5-year survival rates ranging from 28% to 54%. Factors like age, tumor size, and histologic grade influence outcomes. BA is challenging to diagnose and treat, requiring a multidisciplinary approach involving surgeons, oncologists, radiologists, and plastic surgeons. Further research is needed to establish standardized treatment guidelines and improve outcomes for patients.