Vogt-Koyanagi-Harada disease (VKH) is a systemic autoimmune disease typically characterized by a bilateral granulomatous panuveitis associated with extraocular symptoms and neurological, dermatological, and hearing disturbances. The VKH disease is a significant cause of noninfectious uveitis, and its incidence varies based on geography and ethnicity. Women are affected more often than men (2:1 ratio). The peak age of incidence is between the second and fifth decades. Several evidence suggest that VKH disease is a process that primarily targets melanocytes in individuals who are genetically predisposed. VKH disease is a widespread, granulomatous inflammation of the uvea that is bilateral, severe, and nontraumatic in nature. This condition is often linked to neurological and auditory symptoms as well as cutaneous disorders, although the specific symptoms can vary depending on the affected region. The clinical features include prodromic phase, acute uveitic or ophthalmic phase, convalescent phase, recurrent chronic phase, and extraocular manifestations. The diagnosis of VKH disease is primarily based on clinical observation, especially in cases that display both ocular and extraocular symptoms. FA (fluorescein angiography), ICG (indocyanine green) angiography, ultrasound, and OCT (optical coherence tomography) may be necessary. The main differential diagnosis is sympathetic ophthalmia, central serous chorioretinopathy (CSC), uveal effusion syndrome, posterior scleritis, sarcoidosis, noninfectious and infectious choroiditis, and intraocular tumors. Treatment should be carried with IV corticosteroids for three days (pulse therapy), followed by oral corticosteroids with a very slow gradual reduction. Aggressive therapy, early detection, very slow tapering of oral steroids, and the use of immunosuppressants are the key to maintaining good visual acuity.