We describe a 28-year-old man with presumed VKH syndrome, whose presenting symptoms were bilateral impaired vision and headaches. Orbital MR imaging findings included bilateral choroidal and retrobulbar contrast enhancement, while brain findings included white matter abnormalities on FLAIR and leptomeningeal enhancement. Pachymeningeal enhancement has been described previously; herein, we report a patient with VKH syndrome presenting solely with leptomeningeal enhancement. Thus, MR imaging may detect early CNS involvement by VKH disease before the onset of neurologic symptoms.