Vogt-Koyanagi-Harada (VKH) is a rare syndrome affecting tissues containing melanocytes. The possibility of its autoimmune pathogenesis is supported by high frequent HLA-DR4 presentation, commonly associated with other autoimmune diseases. Eyes are the main affected organs, resulting in blindness. Brain disease is a late-onset event, and is extremely rare. Here, we are reporting a 57-year-old woman, a known case of VKH syndrome, presenting with brain encephalopathy several decades after the initial presentation. We think this long period between initial presentation and presentation of encephalopathy due to VKH syndrome has not been described before. She was treated with corticosteroids and discharged home with a good general condition.