We report on a 28-year-old man with a 2-year history of Vogt-Koyanagi-Harada disease (VKH), who developed one-and-a-half syndrome, facial hypoesthesia, facial nerve palsy, hearing loss and limb ataxia on the right side. An MRI showed inflammatory lesions in the pons extending into the left middle cerebellar peduncle. Corticosteroid treatment successfully ameliorated his symptoms and lesions. This patient demonstrated the possible association of brainstem encephalitis with VKH.