Background:Echinococcus multilocularis is a rare infestation in the world with a particularly increased incidence mainly in South America, Central Europe and Asia. Progression of alveolar Echinococcosis is more aggressive that can metastasize to lungs, brain and bones however brain involvement is usually rare with an incidence about 1%.Case Description:We report a 23-year-old man with a cerebellar Echinococcosis multilocularis mimicking a metastatic cerebellar tumor. Suboccipital craniotomy was performed for gross total removal of the tumor. Histopathological specimens confirmed the diagnosis of Echinococcosis multilocularis.Conclusion:Radical surgical excision should be recommended for single Echinococcosis multilocularis lesions particularly at infratentorial localization.