Pancreatic encephalopathy refers to a gamut of neuropsychiatric symptoms complicating acute pancreatitis. Osmotic myelinolysis is a known complication of pancreatic encephalopathy. We evaluated a 58-year-old woman with pancreatic encephalopathy associated to pontine and extrapontine myelinolysis involving the brain and spinal cord. To our knowledge, this is the first clinic pathological case report of pancreatic encephalopathy involving the spinal cord.