Hereditary hemorrhagic telangiectasia (HHT) is a vascular disease with multiple organ manifestations. Severe hepatic involvement is an unusal indication for liver transplantation. We report on a 39-year-old woman diagnosed with HHT and decompensation of the disease during her second pregnancy. After delivery, hemobilia occurred, followed by severe therapy-resistant cholangitis and progressive liver dysfunction. Because of progressive loss of liver function, orthotopic liver transplantation needed to be performed. The various aspects of liver involvement in Osler's disease, diagnosis, and therapy are discussed.
