Background: Cogan's anterior internuclear ophthalmoplegia (INO) is characterized by INO with inability to converge and commonly thought to be due to rostral midbrain lesion. A lesion outside midbrain that causes unilateral Cogan's anterior INO combined with upgaze palsy and ataxia are rarely described.Case Presentation: A 67-year old male presented with left Cogan's anterior internuclear ophthalmoplegia (INO), left appendicular ataxia and bilateral upgaze palsy.