Bickerstaff's brainstem encephalitis (BBE) is a rare diagnosis different from meningoencephalitis with ophthalmoplegia, ataxia and confusion. Less than 100 cases have been described in the literature. We described the fatal case of a 69-year-old man who presented a severe consciousness disorders, distal weakness in the four limbs, and the BBE triad a few days after his admission for pneumonia.