Posterior reversible encephalopathy syndrome (PRES), a rare disease is characterized by multiple neurological complications. It has been reported to be associated with the use of angiogenesis inhibitors such as sorafenib, sunitinib, pazopanib, regorafenib, and lenvatinib. We reported a case of 76-year-old woman with history of stage III hepatocellular carcinoma (HCC), who developed adverse drug reactions related to pazopanib induced PRES. Pazopanib, an angiogenesis inhibitor which inhibits the vascular endothelial growth factor (VEGF) pathway may lead to vascular endothelial damage, and these pathophysiological changes may lead to vascular leaks and brain edema. Medical staff must be aware of the possible association between angiogenesis inhibitors and the development of PRES. In patients with retroperitoneal soft-tissue sarcoma undergoing treatment with pazopanib, regular monitoring of their blood pressure and following-up brain magnetic resonance imaging (MRI) should be encouraged.