Abstract: Budd-Chiari syndrome （BCS） is a clinical syndrome of portal vein and/or inferior vena cava （IVC） hypertension caused by obstruction of the hepatic veins and/or the IVC proximal to its opening. Previous studies have shown that there are significant differences in the etiology， clinical classification， and therapeutic strategy of BCS across different regions. In Western countries， BCS is often secondary to thrombotic disorders， especially myeloproliferative neoplasms， with hepatic vein obstruction as the main lesion； the treatment of BCS mainly follows a stepwise strategy of anticoagulation， recanalization， shunting， and liver transplantation， with relatively early timing for transjugular intrahepatic portosystemic shunt. In contrast， BCS in China mainly involves membranous obstruction of the retrohepatic IVC and mixed-type obstruction， and percutaneous transluminal angioplasty is the core treatment method for BCS. These differences may be associated with various factors such as genetic background， environmental exposure， and healthcare practice patterns， with a significant impact on clinical decision-making and prognostic assessment. This article systematically summarizes the geographic heterogeneity of BCS through a literature review， so as to provide a cross-regional diagnostic and therapeutic reference for clinicians， as well as clues and directions for international collaborative research on the regional differences of BCS in the future.