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A five year old boy manifested with sudden onset of left sided hemiplegia with urinary and fecal incontinence. CT Scan of head revealed evidence of cerebral infarction and magnetic resonance angiography revealed bilateral internal carotid artery stenosis with extensive collateral channels. Ten days later the patient developed typical symptoms of Kluver Bucy syndrome with hyperorality, hypersexuality, loss of memory and hypermetamorphosis. This is the first case report of association of Moya Moya disease with Kluver Bucy Syndrome, Presumably due to involvement of the anterior portion of the limbic system.