Abstract
Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction, frequently due to an underlying thrombophilic disorder. Acute myeloid leukemia rarely presents as acute BCS due to hyperfibrinolysis, hyperleukocytosis, nonspecific proteolytic activity, and disseminated intravascular coagulation causing acute hepatic vein thrombosis. In patients presenting with acute BCS with acute liver failure (ALF), a high index of suspicion and exclusion of underlying malignancy is a must, as it is a contraindication for liver transplantation. We report a case of a 19-year-old Caucasian male who presented with acute BCS causing ALF as an initial presentation of acute myelogenous leukemia.