Primary angiosarcoma (PAS) of the breast that has not received any previous radiotherapy is a rare and highly aggressive tumor with the diagnostic pitfall of a high histological differentiation. Surgical resection is the primary treatment for these tumors. Wide excision of the tumor or mastectomy is the gold standard of treatment, but there is a high risk for locoregional and distant recurrences. Here we present a case of a 47-year-old premenopausal woman with PAS of the breast masquerading as hemangioma on the first core biopsy. One month after wide excision of the tumor, local recurrence occurred and another 3 months later disseminated metastases were diagnosed in the lungs and in the spine of the patient.