Brain stem encephalitis is a rare disease, which can be triggered by infection, with an unclear etiology, in which infectious and autoimmune mechanisms are thought to play a role in the pathogenesis . Major clinical symptoms are ataxia, muscle weakness and ocular and bulbar dysfunction . Its etiology can vary from infection to autoimmune reasons. Listeria, enterovirus type 71 and Herpes simplex virus are the most common infectious causes. It may also be caused by neurobehcets, Bickerstaff's encephalitis, autoimmune diseases such as multiple sclerosis and paraneoplastic reasons [3-5]. With those in mind, the underlying etiology is unclear in most cases . Treatment is based on etiology. Immunosuppressive treatment can be tried in brain stem encephalitis patients whose etiology can not be made clear . In this case report, we present a case of brainstem encephalitis which did not respond to the present treatment and was treated with plasmapheresis.