Vogt-Koyanagi-Harada syndrome (VKH) is a bilateral intraocular granulomatous panuveitis which is frequently associated with systemic manifestations such as meningismus, tinnitus, poliosis and vitiligo of autoimmune aetiology. Headache by itself, does not fulfill the diagnostic criteria and is insufficient for the diagnosis. A 22-year-old male presented with a 10 day history of headache, followed by decreased vision in both eyes. Slit lamp biomicroscopy revealed sluggishly reactive pupils with anterior uveitis, mild vitritis and hyperaemic discs with bilateral exudative retinal detachments. All uveitis workups were negative. Follow up of three years revealed no neurological or auditory symptoms. Headache alone, followed by decreased vision, before the onset of neurological and auditory symptoms, can be an initial presentation of Vogt-Koyanagi-Harada (VKH) syndrome. VKH should be considered in the differential diagnosis of atypical presentations of headache.
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