Hemodialysis-related portal-systemic encephalopathy (HRPSE) is characterized by the presence of portosystemic encephalopathy without liver dysfunction, usually caused by changes in the systemic venous flow related to hemodialysis. We herein describe the case of a 75-year-old woman who developed hepatic encephalopathy five years after the initiation of hemodialysis. Abdominal contrast-enhanced computed tomography (CT) and three-dimensional CT angiography revealed a portosystemic venous shunt, and the patient was diagnosed with portosystemic encephalopathy. Occlusion therapy ameliorated her disturbance of consciousness. HRPSE should be recognized as a treatable neuropsychiatric disorder.