Alveolar echinococcosis is a parasitic disease limited to the northern hemisphere. The disease occurs primarily in the liver and shows a profile mimicking slow-growing malignant tumors. Echinococcus multilocularis infection is fatal if left untreated. It can cause several complications by infiltrating the vascular structures, biliary tracts, and the hilum of the liver. As it can invade the adjacent organs or can spread to distant organs, alveolar echinococcosis can easily be confused with malignancies. We provide a brief review of epidemiologic and pathophysiologic profile of alveolar echinococcosis and clinical features of the disease. This article focuses primarily on the imaging features of alveolar echinococcosis on ultrasonogra-phy, computed tomography, magnetic resonance imaging, diffusion-weighted imaging and positron emission tomography-computed tomography. We also reviewed the role of radiology in diagnosis, management, and follow-up of the disease.