Abstract: Angiosarcoma(AS) is a rare malignant soft tissue sarcoma with poor differentiation and outcome originating from vascular or lymphatic endothelial cells. Currently, there is still no consensus on the treatment of AS. The chemoradiotherapy and surgical resection are the main treatment, but the curative effect is not good. With the rapid development of molecular biology, new molecular targets have been found gradually, which can benefit some patients. In addition, with the development of immunotherapy, the treatment of AS has been greatly enriched. This article expands on the progress of molecular targeting and immunotherapy of angiosarcoma and provides a reference for clinical colleagues.