Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma in late adult life. Unfortunately, advanced MFH has a poor prognosis due to a lack of effective drugs. We present here a case of advanced MFH with partial response to apatinib, a new potent oral small-molecule tyrosine kinase inhibitor targeting the intracellular domain of vascular endothelial growth factor receptor 2 (VEGFR-2). To the best of our knowledge, this is the first case report using apatinib for MFH. 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 the crucial role of VEGFR-2 for MFH. Apatinib could be a new option for the treatment of MFH. Further studies are needed to optimize the treatment.