Bickerstaff 's brainstem encephalitis (BBE) is characterized by acute ophthalmoplegia and ataxia. Some patients with BBE show mental changes, pyramidal signs and sensory disturbance. A forty-three year old woman was diagnosed as BBE in 1996. In 2000, she admitted again with complaints of ataxia and ophthalmoplegia. She was diagnosed as recurrent BBE and an overlap of Guillain-Barre syndrome (GBS). This is the first case of an overlap of BBE and GBS, as well as BBE in Korea.