Cryptococcus neoformans is an opportunistic fungus with a predilection for infecting the meninges. Ocular sequelae of cryptococcal infections of the CNS usually include cranial nerve palsies or papilledema secondary to increased intracranial pressure. Intraocular cryptococcosis occurs less frequently, and over the last 23 years, only 27 cases have been reported, including the case presented here. Intraocular infection was most often manifested by chorioretinal lesions and vitritis. Underlying diseases were detected in only 11 (41%) of the 27 patients. Of note, ocular lesions preceded symptomatic meningitis in six (27%) of 22 patients with CNS involvement. For seven patients, the diagnosis was made by histologic examination of specimens of aqueous or vitreous humor; for another eight patients, the diagnosis was made after enucleation or at autopsy. Ocular involvement frequently led to severe visual loss; return of vision to normal was unusual. Early recognition and treatment may improve outcome for these patients.