Acanthamoeba keratitis (AK) is a potentially devastating infection of the ocular surface caused by amoebas of the genus Acanthamoeba. Although the organism is classically known for being difficult to detect and treat, recent advances in the field have greatly improved diagnostic accuracy and treatment efficacy. In this update, we review the current body of knowledge about AK epidemiology and pathogenesis, discuss the advances in diagnosis with confocal microscopy and polymerase chain reaction, and explore potential novel treatments such as voriconazole, miltefosine, topical steroids, phototherapeutic keratectomy, cross-linking and photodynamic therapy.