Alveolar echinococcosis of the liver is a disease caused by the intrahepatic growth of Echinococcus multilocularis larvae. In contrast to hydatidic cyst caused by Echinococcus granulosus, this is a rare disease in Spain. We present a case of alveolar echinococcosis of the liver which presented with jaundice and hepatomegaly. An infiltrative solid tumor was diagnosed by US and CT scan. The patient required left hepatectomy and interventional radiology procedures with satisfactory results. Diagnosis and treatment of this disease are discussed.