Vogt–Koyanagi–Harada (VKH) disease, also known as uveomeningitic syndrome, is a multisystem autoimmune disorder directed against any melanocyte-containing organ, including the eye, central nervous system, auditory, and integumentary system. We present a case of an African woman with total deafness and blindness of VKH, and discuss the histological characteristics, and review the classification and the management of VKH. The most important factor for prognosis is the immediate treatment. The rarity of this syndrome makes its diagnosis a challenge and the presence of deafness, tinnitus, and vertigo must be considered in its diagnosis.