Budd-Chiari syndrome (BCS) is a hepatic venous outflow block generally resulting from disorders affecting hepatic venous system. Elevated hepatic venous pressure results in portal hypertension. BCS may also cause hepatic encephalopathy. Echinococcus multilocularis is a tapeworm parasite and the natural course of the disease may affect liver parenchyma as well as hepatic venous tree. It is the most terrible parasitic disease of the liver and is easily confused with hepatic malignancies. Albendazole therapy may suppress disease progression. Alveolar echinococcosis of the liver rarely causes Budd-Chiari syndrome-related hepatic encephalopathy (HE). We report a rare case of alveolar echinococcosis-related BCS with HE, who was successfully managed by rifaximin and albendazole.