Budd-Chiari syndrome is a rare and potentially fatal disorder characterized by obstruction or narrowing of the hepatic veins and inferior vena cava. The acute form of the disease often leads to rapid liver failure. In Asia, membranous obstruction of the hepatic portion of the inferior vena cava and localized stenosis at the hepatic venous origin are more common, while in Western countries, hepatic vein obstruction due to underlying thrombotic conditions is more prevalent. Treatment strategies vary and depend on disease severity and location. Anticoagulation therapy is the standard treatment in Western countries but is less commonly used in Japan where catheter-based interventions, such as recanalization, balloon dilation, and stenting, are more frequently employed. We report the case of a 51-year-old man diagnosed with Budd-Chiari syndrome after the treatment of esophageal varices. He initially responded well to nafamostat and balloon dilation, maintaining stability for 8 years, but later developed acute complications, including venous thrombosis and liver failure, despite previously stable follow-up. The patient succumbed to his condition despite aggressive treatment, highlighting the importance of early detection and intervention. This case underscores the need for clearer guidelines on anticoagulation therapy in Japan and emphasizes early management to improve prognosis.