A 41-year-old woman was admitted to the hospital because of diarrhea followed by progressive weakness of all extremities and dysphagia. On neurological examination, she showed facial diplegia, bulbar palsy, flaccid quadriplegia, and absence of all deep tendon reflexes in addition to Laségue's sign. The Campylobacter jejuni Penner type 4 was isolated from the culture of stool. The test of anti-GM1b antibody (IgG) was positive in the serum. The protein content was elevated in the cerebrospinal fluid without pleocytosis. The studies of motor nerve conduction velocity showed a pattern of the axonal neuropathy. This is a case of Guillain-Barré syndrome presenting with the axonal neuropathy possibly due to the immune response directed to GM1b which is triggered by the Campylobacter jejuni Penner type 4 infection.