Alveolar echinococcosis is a parasitic disease which is relatively rare in humans. It almost exclusively affects patients of rural origin living in enzootic regions (Eastern France, Auvergne) and most lesions are in the liver. Several anatomico-clinical forms have been described; the most frequent is the multilocular form, but the disease may consist of one single cyst or abscess. The liver structure is always deeply altered, with compression, inflammation or superinfection. Jaundice, liver enlargement, abdominal pain or signs of secondary localizations are manifestations that lead to the discovery of hepatic alveolar echinococcosis. The anatomical features of the lesions are demonstrated by ultrasounds and computerized tomography. The main differential diagnosis is tumoral pathology of the liver. In most cases the diagnosis of echinococcosis is confirmed by serological tests, although needle or even surgical biopsy might be necessary. Diagnosed at an early stage, alveolar echinococcosis can be amenable to surgical treatment (hepatectomy), and liver transplantation may even be performed. Medical treatment with benzimidazoles seems to be promising.