Ocular manifestations of Whipple's disease result from CNS involvement, direct intraocular involvement, or both. Ocular signs caused by CNS involvement occurred in 30 of the 34 patients with ocular manifestations described in the literature. In only four of the 34 cases were ocular manifestations due solely to intraocular involvement. Two cases reported herein had unique intraocular involvement, manifested as diffuse chorioretinal inflammation. Fluorescein angiography showed diffuse vasculitis, with hemorrhages, exudates, retinal capillary occlusion in the perifoveal and midperipheral areas, capillary dilation at the optic disc, and choroidal folds. Proper antibiotic treatment resulted in ocular and systemic recovery. Follow-up is mandatory because of possible recurrences, which may start with intraocular signs, followed by systemic manifestations. Some cases progress to fetal CNS involvement. Therefore, when intraocular signs recur in treated patients, antibiotic therapy should be restarted or dose should be increased.