A 59-year-old Asian woman suffered from a TCH followed by sudden, binocular blurred vision, mimicking pituitary apoplexy. The diagnostic workup (including fluorescein angiography, MRI of the brain, and CSF analysis) showed severe optic disc swelling and dye leakage of multiple faint hyperfluorescent spots at retinal pigment epithelium level, diffuse pachymeningeal hypertrophy, and monocytic pleocytosis, respectively. VKH disease should be considered in the differential diagnosis of patients presenting with a TCH followed by sudden, bilateral decreased visual acuity.