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Acanthamoeba keratitis is a potentially blinding microbial disease that has been increasing in incidence during the past two decades. Prognosis of this serious disease had been dismal, but improvement in diagnosis, a better understanding of the natural course of the disease, and recent introduction of multiple and effective therapeutic agents have resulted in improvement of visual outcomes.A review of literature pertaining to Acanthamoeba keratitis.Contact lens wear and exposure to contaminated water sources remain the most important risk factors; however, in vivo confocal microscopy and improved biomicroscopic screening have proven instrumental in accurate early diagnosis. Complications of Acanthamoeba keratitis include dacryoadenitis, corneal melting and scarring, severe secondary glaucoma, cataract, and chronic anterior segment inflammation that can rarely lead to reactive blinding retinal ischemia. Combination chemotherapeutic agents have been shown to be more effective than monotherapy, whereas rehabilitative surgery such as penetrating keratoplasty is best performed on a quiet eye free of ocular inflammation and with no residual amoebae.Increased suspicion by clinicians for Acanthamoeba and confocal microscopy have allowed more rapid and accurate diagnosis; treatment with multiple antiamoeba drugs is essential to disease resolution. Provided there are no residual amoebae after treatment, penetrating keratoplasty has been successful in visual rehabilitation. Secondary glaucoma occurs frequently and may require drainage procedures for control of intraocular pressure. Posterior complications are rare but may lead to ischemic retinitis.