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Alveolar echinococcosis is a much-feared parasitic zoonosis caused by the larval stage of Echinococcus multilocularis. Mainland Norway is free from infection, but alveolar echinococcosis is, on rare occasions, imported from endemic regions. Those infected develop slow-growing, multicystic tumours that are clinically and radiologically reminiscent of malignant disease. The disease mainly attacks the liver. Treatment often consists of extensive surgical resection in combination with prolonged use of albendazole. In this clinical review article we summarise the life cycle, clinical findings, diagnosis, treatment and epidemiology of alveolar echinococcosis, and provide examples of the disease course with two patient case reports.