Budd-Chiari syndrome is a spectrum of disease states, including anatomic abnormalities and hypercoagulable disorders, resulting in hepatic venous outflow occlusion. Clinical manifestations observed in the majority of patients include hepatomegaly, right upper quadrant pain, and abdominal ascites. This article outlines the approach to clinical diagnosis and supportive medical therapy in patients who have BCS and reviews the clinical data supporting surgical shunting and liver transplantation as viable treatment options in this patient population.