Amoebae of the genus Acanthamoeba Volkonsky, 1931 are ubiquitous, amphizoic organisms with a cosmopolitan distribution. Pathogenic strains are the causative agents of a difficult to treat disease, granulomatous amoebic encephalitis (GAE), and skin infections in immunocompromised individuals, and of a painful corneal disease--amoebic keratitis (AK) in immunocompetent individuals. The major portals of entry are the nasopharyngeal mucosa, pulmonary parenchyma, skin lesions (GAE, skin infections), eyes in contact lenses wearers with a history of improper contact lens wear and care, or corneal trauma (AK). Symptoms of the diseases are non-specific and variable which alongside with the lack of awareness among health care professionals often hamper early diagnosis. While treatment options for GAE and skin infections are limited and poorly effective, various antifungals and antimicrobials have proved beneficial in AK, although the therapy is often complicated and long.