A 36-year-old man with pharyngeal-cervical-brachial variant of Guillain-Barré syndrome (PCB) was described. Neurologic examination revealed total ophthalmoplegia, pharyngeal-cervical-brachial weakness and hyporeflexia in the upper limbs, sparing power and tendon reflexes in the lower limbs. Enzyme-linked immunosorbent assay showed that he had high titer of IgG antibody to GT1a (1:32,000), which did not cross-react with GQ1 b or GD1a. Thin-layer chromatography immunostaining confirmed that his serum IgG reacted with GT1a. These findings show that IgG anti-GT1a antibody without cross-reactivity with GQ1b plays a role in the development of PCB.