Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Rendu-Weber disease, is a dominant disorder of the long arm of chromosome 9 in humans with an incidence of about 1 to 2 per 100,000, with no geographical or racial differences. It occurs in the skin, lungs, gastrointestinal tract and brain, with pulmonary arteriovenous malformations in 60% to 70% of HHT patients [3] and less frequently in the liver, accounting for 8% to 31% of the total incidence. There are at least two genetic mutations associated with the development of HHT, the ENG mutation that causes HHT type 1, in which pulmonary arteriovenous malformation occurs in more than 40% of patients, and the ALK1 mutation that causes HHT type 2, in which pulmonary arteriovenous malformation occurs in 14% of patients. Liver involvement was found to be caused mostly by ALK-1 gene variants, while ENG gene variants were rare. The pathology of liver-involved HTT is characterized by diffuse intrahepatic vascular malformations, including three types: hepatic artery-venous short circuit, hepatic artery-portal vein short circuit, and portal vein-hepatic vein short circuit. Patients with HHT are characterized by dilated capillaries and microvenules in the skin, mucous membranes and viscera with hemorrhage, so patients with recurrent rhinorrhea and dilated skin capillaries should be highly suspected of HHT, especially in patients with combined abdominal pain and jaundice. HHT involving the liver. further clinical examination of gastrointestinal microscopy, cranial, pulmonary and hepatic revascularization to fully understand the presence of visceral vascular malformations and, if necessary, to examine their family ALK-1 and ENG genes. No specific treatment is required for HHT patients with asymptomatic liver involvement, but they should be reviewed regularly and followed up closely. For patients with symptomatic HHT with liver involvement, treatment should be started as early as possible.1) Symptomatic treatment: The treatment for complications of high blood volume heart failure and portal hypertension is the same as that for other causes of heart failure and portal hypertension. Patients with high-volume heart failure should receive intensive drug therapy, including correction of anemia and arrhythmias that predispose to heart failure. It should be noted that transjugular intrahepatic portosystemic shunt (T IPS) is not effective in improving upper gastrointestinal bleeding symptoms in patients with HHT with liver involvement. Abdominal pain due to biliary ischemia is mainly treated with analgesia, and broad-spectrum antibiotics should be added when symptoms of cholangitis are present. Because of the presence of biliary ischemia, patients with biliary symptoms of HHT are not recommended to undergo dye-infused surgery. It is recommended as the first choice for non-pharmacological treatment. For patients with HHT with hepatobiliary ischemia, embolization or hepatic artery ligation is not recommended because it may aggravate the ischemia and lead to fatal complications such as bile duct necrosis and hepatic necrosis. 3) Liver transplantation: Liver transplantation is the only proven curative treatment option for HHT with liver involvement, but the high cost, lack of donors, and lifelong immunosuppressive therapy make it difficult to be the preferred option for HHT with liver involvement.