Hepatic hereditary hemorrhagic telangiectasia (HHHT) accounts for 47–74% of hereditary hemorrhagic telangiectasia (HHT) patients. HHT is an autosomal dominant hereditary vascular dysplasia disease with the main pathological changes being a lack of elastic fibers and smooth muscles in the affected blood vessels, damaged integrity of the tube wall and the susceptibility to bleeding, and the lack of contractility that leads to capillary dilatation, angiomatoid dilation, and arteriovenous malformations, which can affect any organ and system. Repeated bleeding and anemia resulting from capillary dilation in the skin and mucosa and multiple visceral arteriovenous malformation are the clinical characteristics of HHT, which may can cause liver cirrhosis and hepatic encephalopathy. There is currently no specific data on the epidemiological features of HHHT. CT/MRI plain scans were mostly negative in patients with HHHT, while multiphase enhanced scans or CTA can show characteristic imaging findings, including diffuse vascular abnormalities (DVAL, such as multiple telangiectasia, hemangiomatous dilatation and arteriovenous malformation, hepatic arteriovenous fistula, portal vein hepatic venous fistula, etc.), focal hepatic lesions, intrahepatic bile duct abnormalities, and hepatic fibrosis. It is significantly important for early diagnosis to improve awareness and be familiar with imaging findings of HHHT.