LD (with its ocular manifestations) is a worldwide disorder that is increasing in frequency. It is a treatable multisystemic disease that presents in three stages of severity. It can present with unusual forms of conjunctivitis, keratitis, cranial nerve palsies, optic nerve disease, uveitis, vitreitis, and other forms of posterior segment inflammatory disease. A patient with any of these ocular manifestations should be questioned for exposure to an area endemic for LD, tick bites, skin rash, or arthritis. Such patients should undergo serological testing. If the clinical presentation is suggestive of LD, a course of oral antibiotics should be used (unless the patient gives a history of adequate therapy). Topical corticosteroids can be used for anterior segment inflammation. An antibiotic therapeutic trial can be used for posterior segment or neuroophthalmic disease. Systemic corticosteroids without concomitant antibiotics should not be used in the treatment of ocular LD. If ocular LD is discovered and treated early, response to therapy usually is satisfactory.