Angiosarcoma (AS) is a rare malignant tumor of the skin and soft tissues. Due to this lesions's histopathologic similarity to other soft tissue and skin neoplasms, several names had been previously used to describe AS. In the following article, we present a comprehensive review of the literature and prior studies pertaining to AS. The purpose of this article is to provide the reader with a better understanding of the causative factors, clinical findings, and histological features of this uncommon but potentially lethal tumor. Furthermore, we discuss present therapeutic options and outcomes as well as summarize recent advances in AS tumor biology and cytogenetics.