Alveolar echinococcosis (AE), or alveolar hydatid disease (AHD), is an uncommon parasitic disease which behaves like a malignant tumor of the liver, producing liver necrosis and invading the biliary tract, main hepatic vessels and surrounding structures. Only 25% of the patients are candidates for radical surgical resection, the only currently available definitive treatment. Ortho-topic liver transplantation (OLT) has been performed for terminal AE since the mid-1980s. This article reviews the specific problems related to OLT in AE. They represent a number of relative contraindications. Invasion of the duodenum and pancreas or the right atrium by the parasitic masses, and cerebral metastases, are absolute contraindication to OLT. Today, OLT can be considered in AE patients with chronic Budd-Chiari syndrome, severe secondary biliary cirrhosis, intractable cholangitis and biliary sepsis, or involvement of both lobes where radical resection is not possible. In our series, which comprises 22 of the 28 European patients transplanted for AE, 1- and 5-year actuarial survival are 78 and 65% respectively.