The typical clinical appearance of acanthamoeba keratitis includes pseudodendritic epitheliopathy, perineuritis, ring infiltrates or multifocal stromal infiltrates and in some cases limbitis with infiltration of the conjunctiva and/or sterile anterior uveitis. In 83-93 % of cases of acanthamoeba keratitis the patients were contact lens wearers. Acanthamoeba keratitis is diagnosed by polymerase chain reaction (PCR), confocal biomicroscopy, in vitro cultivation and histopathological examination. Information on reliability and efficacy of conservative and surgical therapy of acanthamoeba keratitis has only been published in case series but not yet verified through randomized controlled clinical studies. After early diagnosis acanthamoeba keratitis can often be successfully treated using triple topical therapy with polyhexamide, propamidine isethionate and neomycin. Topical therapy should be continued for up to 1 year. In therapy-resistant cases cryotherapy, amniotic membrane transplantation, crosslinking therapy and therapeutic keratoplasty can be performed. The prognosis of keratoplasty following acanthamoeba keratitis is more favorable if there were no signs of infection at least 3 months before surgery.