We report the clinical course, and neurophysiological and neuroimaging findings of a patient with Guillain-Barré syndrome associated with central nervous system lesions. During a course of intravenous immunoglobulin therapy, she had headache with meningism. Cerebral magnetic resonance imaging showed lesions in both frontal and right occipital lobes. Cerebrospinal fluid showed a raised protein concentration accompanied by mild pleocytosis. Her symptoms resolved within two months. Subsequent magnetic resonance imaging revealed cavity formation in the deep white matter and atrophic changes in the right occipital lobes.