Amoebas from the genus Acanthamoeba are cosmopolitan organisms, which can exist as free-living organisms and as parasites within host tissue. Acanthamoeba infection present a serious risk to human health and are characterized by high mortality, especially in immunocompromised individuals. These protozoa are the etiological factors of granulomatous amoebic encephalitis (GAE) and Acanthamoeba keratitis (AK). They can also live in the lungs, adrenals glands, nose, throat, and bones of the host. Furthermore, the amoebas can be vectors of pathogenic bacteria. Acanthamoeba infection caused is a serious clinical problem mainly due to limited progress in diagnostics and treatment of this infection, which is associated with insufficient knowledge of pathogenesis, pathophysiology and the host immune response against Acanthamoeba antigens. This review study presents the biology of Acanthamoeba sp. as well as pathogenicity, diagnostics, and treatment of amoebas infections. It also presents data, including experimental results, concerning pathogenic properties and the host’s immunology response against Acanthamoeba sp.