Alveolar echinococcosis (AE) is an uncommon parasitic disease that is caused by the intrahepatic growth of Echinococcus multilocularis larvae. Eastern France is endemic for AE. A wild cycle allows the parasite to subsist in nature. The adult worm develops in the small intestine of foxes. The eggs of the taenia are dispersed on the ground by the foxes' stools. Rodents are infested by eating contaminated vegetables and larvae develop in the liver. Humans, accidental intermediate hosts, become infested due to either eating contaminated wild fruits, or by touching foxes or dogs. The human lesions of AE are often compared to a liver cancer which progress very slowly, causing necrosis and fibrosis. Complications invariably occurred during the follow-up. The most frequent are biliary complications, bleeding related to portal hypertension or Budd Chiari syndrome. During the last years, important progress has been made in many fields concerning AE, particularly in the immunology, epidemiology and therapy of this disease. The sole efficient treatment is partial hepatectomy. Recently, liver transplantation have been successfully performed for incurable AE.