A 40-year-old man developed obstructive hydrocephalus of uncertain cause followed by bilateral intraocular inflammation, which was initially diagnosed as toxoplasmic retinochoroiditis. When visual acuity in the left eye decreased to no light perception and vision in the right eye was failing, a diagnostic enucleation of the blind left eye was performed. A diagnosis of cryptococcal endophthalmitis was established and the remaining right eye showed improvement after systemic anticryptococcal therapy.