Vogt–Koyanagi–Harada (VKH) syndrome is an idiopathic systemic inflammatory disorder that affects various melanocyte-containing structures, including the eyes, meninges, inner ear and skin. It classically leads to bilateral chronic granulomatous diffuse uveitis, and its extraocular manifestation can include sensorineural hearing loss, meningitis, and cutaneous findings of vitiligo, poliosis (loss of hair pigment) and alopecia. Ethnicity/racial background and HLA genotype play a strong role in the pathogenesis of VKH syndrome. The inflammatory process is not completely understood, but recent studies have pointed to several potential autoantigen targets, and have also demonstrated the role IL-23 plays in inducing the differentiation of Th17 cells and the subsequent production of IL-17. The success in preserving the vision of VKH syndrome patients hinges on early diagnosis and aggressive treatment that includes immunomodulatory therapy, and as a result ophthalmologists are increasingly referring such patients to rheumatologists for management. It is therefore necessary for the rheumatologists to be familiar with VKH syndrome and its clinical aspects and management. Although the role of biologics in the treatment of VKH syndrome has yet to be investigated, it is possible that such treatments may prove beneficial, given what is currently known about the pathogenesis of the disease.