Angiosarcoma of the temporal bone is an extremely rare malignant tumor, which originates from vascular endothelium. We describe a case of a 57-year-old woman, initially presenting with progressive hearing loss, otorrhea as well as facial palsy diagnosed as a mastoiditis elsewhere. After subtotal mastoidectomy histological examination revealed an angiosarcoma of the mastoid. Since the tumor was not completely removed lateral petrosectomy and postoperative radiotherapy were performed. Afterwards the patient developed local recurrence with intracerebral tumor extension. During palliative polychemotherapy the patient developed a pneumonia and deceased. In this manuscript the morphology, imaging characteristics and current treatment options of angiosarcomas of the lateral skull base are reviewed and discussed.