The diagnosis of ocular toxoplasmosis is based most often on the presence of characteristic clinical findings, which include focal retinochoroiditis, an adjacent or nearby retinochoroidal scar, and moderate to severe vitreous inflammation. However, a variety of less common, “atypical” presentations may be unfamiliar to clinicians, delaying both diagnosis and treatment. Patients who are immunocompromised or elderly may, for example, present with large, multiple and/or bilateral lesions. Other unusual manifestations include punctate outer retinal toxoplasmosis, retinal vasculitis, retinal vascular occlusions, rhegmatogenous and serous retinal detachments, a unilateral pigmentary retinopathy mimicking retinitis pigmentosa, neuroretinitis and other forms of optic neuropathy, and scleritis. Although in the past most cases of ocular toxoplasmosis were considered to result from reactivation of a congenital infection, it is now believed that postnatally acquired infection accounts for many cases of this disease. With appropriate use of antiparasitic therapy, the visual prognosis for patients with both typical and atypical forms of ocular toxoplasmosis may be good.