Infantile hepatic hemangioendothelioma (IHHE) is a rare vascular tumor that presents before the age of 6 months. The patients with IHHE suffer from high-output congestive heart failure caused by major arteriovenous fisutulas in the liver, which leads to respiratory compromise and results in a high mortality rate despite medical treatments. A case of 4-month-old baby with liver failure caused by IHHE is reported. The baby received an urgent liver transplantation from a living donor. A monosegmental graft was used to mitigate graft-to- recipient size mismatching. The surgical procedure of monosegmental living donor liver transplantation also is discussed.