Angiosarcoma is a rare and aggressive vascular malignancy with a poor prognosis. There is no standard chemotherapy regime for advanced angiosarcoma. Here, we report a case of advanced angiosarcoma that was successfully treated with apatinib, an oral tyrosine kinase inhibitor targeting the intracellular domain of vascular endothelial growth factor receptor-2 (VEGFR-2). To our knowledge, this is the first case to report the successful use of apatinib for angiosarcoma. Furthermore, the administration of apatinib results in fewer toxic effects than traditional cytotoxic chemotherapy. Quantitative polymerase chain reaction analysis revealed high expression of VEGFR-2 mRNA, suggesting that apatinib leads to clinical response by inhibiting VEGFR-2 tyrosine kinase activity and that VEGFR-2 plays a crucial role for angiosarcoma. Apatinib may be an additional option for angiosarcoma especially for the aged and patients with poor performance status. Further prospective studies are required to optimize the use of apatinib in patients with angiosarcoma and to identify which patients will benefit from the agent.