VKH disease is an uncommon granulomatous inflammatory disorder characterized by bilateral panuveitis associated with neurological and cutaneous abnormalities.

VKH disease occurs worldwide; it accounts for 1-4 % of endogenous uveitis in the United States and as many as 9 % of cases in Japan. It primarily affects darkly pigmented groups, including Asians,Native Americans, Indians. The exact etiology of VKH is still unknown. An autoimmune process in a genetically susceptible individual who contacts a viral infection is the most likely mechanism.The autoimmun aspect in VKH includes a cellular immune response against melanocytes. A genetic role in VKH is strengthened by the reports of familial cases.The meningeal or neurological manifestations of VKH disease usually occur in the initial stage of the disorder and consist of headache, meningismus, cranial nerve palsies, CSF pleocytosis and occasionally focal neurological signs such as hemiparesis,ataxia .There may be auditory disturbances, including tinnitus and dysacusis,as well as vertigo. Vision loss occurs due to complications of recurrent uveitis such as cataract and glaucoma. Alopecia, vitiligo and poliozis are the typical cutaneous manifestations of the disease. We are reporting a patient diagnosed as VKH syndrome which is rare in Turkey and presenting as uveameningoensefalitis