Hepatic encephalopathy is a common complication of end-stage liver disease. It happens in the presence of significant hepatic dysfunction and the diversion of the portal blood to the systemic circulation (porto-systemic shunts). Hereditary Hemorrhagic Telangiectasia (HHT) or Osler-Weber-Rendu disease, can also involve the liver along with the skin, mucous membranes, lung, brain, and gastrointestinal tract. The prevalence of hepatic involvement in HHT ranges between 41% and 78%. Although most patients with HHT are asymptomatic, some may have symptoms due to high-output heart failure, portal hypertension and biliary disease. Less commonly, patients may also develop porto-systemic encephalopathy. In this case report, we present a woman aged 63 years, who was diagnosed as having hepatic encephalopathy due to a portosystemic shunt.