Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu syndrome is a rare condition which can result in significant systemic and hepatobiliary abnormalities. Liver involvement in HHT consists primarily of the consequence of various intrahepatic shunts. Even though these vascular shunts are present in the majority of patients with HHT, symptoms occur only in minority with clear predilection to female gender. The symptoms and imaging findings of liver vascular malformations can be easily overlooked or misdiagnosed which can result in delay in treatment or potentially harmful vascular interventions. In this case report, we discuss the pathophysiology of HHT in liver involvement, role of imaging in diagnosis, and the possible role of interventional radiologist in the treatment.