Abstract: Cutaneous angiosarcoma is a malignant soft-tissue sarcoma derived from vascular or lymphatic endothelial cells.It is very rare,and mostly affects the scalp and face of the elderly,while cutaneous angiosarcoma occurring in other sites is usually associated with radiotherapy or chronic lymphoedema.Cutaneous angiosarcoma usually has various clinical manifestations and histological features,and is easily misdiagnosed.In addition,it is highly aggressive with high risks of recurrence and metastasis.Its pathogenesis is still unclear,and effective therapeutic approaches are unavailable currently.For patients with early cutaneous angiosarcoma,surgery combined with radiotherapy is the main treatment method.For patients with locally advanced and metastatic cutaneous angiosarcoma,cytotoxic chemotherapy is usually adopted at present.However,the long-term prognosis remains unsatisfactory.Recently,some scholars have started to study the molecular pathogenesis of cutaneous angiosarcoma,and explore related targeted therapy.