We report a case of markedly asymmetric pharyngeal-cervical-brachial weakness. Acute progression of symptoms, albuminocytologic dissociation in cerebrospinal fluid, electrophysiologic evidence of demyelination and elevation of IgG anti-GT1 a antibody titer paralleled the clinical course, support the diagnosis of Guillain-Barré syndrome. Guillain-Barré syndrome should be considered in the differential diagnosis of cranial neuropathy, even in cases where there is marked asymmetry.