Alveolar echinococcosis is a parasitic disease limited to the northern hemisphere. The diseaseoccurs primarily in the liver and shows a profile mimicking slow-growing malignant tumors. Echinococcusmultilocularis infection is fatal if left untreated. It can cause several complications byinfiltrating the vascular structures, biliary tracts, and the hilum of the liver. As it can invade theadjacent organs or can spread to distant organs, alveolar echinococcosis can easily be confusedwith malignancies. We provide a brief review of epidemiologic and pathophysiologic profile ofalveolar echinococcosis and clinical features of the disease. This article focuses primarily on theimaging features of alveolar echinococcosis on ultrasonography, computed tomography, magneticresonance imaging, diffusion-weighted imaging and positron emission tomography-computedtomography. We also reviewed the role of radiology in diagnosis, management, and follow-upof the disease.