Alveolar echinococcosis is an infectious disease caused by Echinococcus multilocularis and it is frequently diagnosed as a space-occupying lesion in the liver. The growth pattern may be similar to that of a malignant tumour with extensive liver infiltration, spreading into neighbouring organs and forming metastasis-like masses in distant organs. Thus, it is one of the differential diagnoses of liver cancer. We report a case that presented as a klatskin tumour clinically and radiologically, but was revealed by pathologic and serologic work-up. Since the courses of these two diseases, a malignancy and an infectious disease, are far beyond comparison, echinococcosis should always be considered in differential diagnosis of obstructive jaundice, especially in the endemic regions.