Alveolar echinococcosis of the liver is a rare larval cestode disease which is due to the intrahepatic growth of the tapeworm Echinococcus multilocularis. This cestode naturally evolves as a larval stage within cysts in the body of carnivores. Humans are accidental intermediate hosts and become infected, either by eating food contaminated with carnivore-originated eggs or by touching foxes. It behaves as malignant liver tumour and rarely causes Budd-Chiari syndrome and variceal bleeding. Budd-Chiari syndrome is a hepatic venous outflow tract obstruction and may be present abdominal pain, hepatomegaly and ascites. Parasitic cysts may cause compression and thrombosis of the hepatic venous outflow tract. It may present as portal hypertension and variceal upper gastrointestinal bleeding. We here in report a 47-year-old woman without a prior history of liver disease presented with Budd-Chiari syndrome and variceal bleeding due to Alveolar echinococcosis. The course of this rare disease is demonstrated by means of the most important laboratory, serologic and radiologic parameters.