Acanthamoeba species are an important cause of microbial keratitis that may cause severe ocular inflammation and visual loss. The first cases were recognized in 1973, but the disease remained very rare until the 1980s, when an increase in incidence mainly associated with contact lens wear was reported. There is an increased risk when contact lens rinsing and soaking solutions are prepared with nonsterile water and salt tablets. The clinical picture is often characterized by severe pain with an early superficial keratitis that is often treated as herpes simplex infection. Subsequently a characteristic radial perineural infiltration may be seen, and ring infiltration is common. Limbitis and scleritis are frequent. Laboratory diagnosis is primarily by culture of epithelial samples inoculated onto agar plates spread with bacteria. Direct microscopy of samples using stains for the cyst wall or immunostaining may also be employed. A variety of topically applied therapeutic agents are thought to be effective, including propamidine isethionate, clotrimazole, polyhexamethylene biguanide, and chlorhexidine. Various combinations of these and other agents have been employed, often resulting in medical cure, especially if treatment is commenced early in the course of the disease. Penetrating keratoplasty is preferably avoided in inflamed eyes, but may be necessary in severe cases to preserve the globe or, when the infection has resolved, to restore corneal clarity for optical reasons.