[b]Abstract Introduction[/b]. Among free-living amoebae that are widely distributed in nature only four genera/species are known as agents of human infections:[i] Acanthamoeba spp., Naegleriafowleri, Balamuthia mandrillaris[/i] and[i] Sappiniapedata[/i]. These amoebae are not well adapted to parasitism, and could exist in the human environment without the need for a host. Infections due to these amoebae, despite low morbidity, are characterized by relatively high mortality rate and pose serious clinical problems. [b]Objectve[/b]. This review study presents and summarizes current knowledge about infections due to pathogenic and opportunistic free-living amoebae focused on epidemiology, clinical manifestations, diagnosis and treatment based on global literature. [b]State of knowledge[/b]. All four genera have been recognized as etiologic factors of fatal central nervous system infections and other serious diseases in humans. [i]N. fowleri[/i] causes an acute fulminating meningoencephalitis in children and young adults. [i]Acanthamoeba spp[/i]. and [i]B.mandrillaris[/i] are opportunistic pathogens causing granulomatous amoebic encephalitis and disseminated or localized infections which could affect the skin, sinuses, lungs, adrenals and/or bones. [i]Acanthamoeba spp[/i]. is also the main agent of acute eye infection -[i] Acanthamoeba keratitis, [/i]mostly in contact lens wearers. However, there is only one recognized case of encephalitis caused by [i]S. pedata. [/i] [b]Conclusions[/b]. Amoebic diseases are difficult to diagnose which leads to delayed treatment, and result in a high mortality rate. Considering those issues, there is an urgent need to draw more attention to this type of diseases.