Peripheral neuropathy associated with hyper-IgE-emia have been rarely reported. Here we present a 72-year-old man with acute motor axonal neuropathy who had relatively poor prognosis. The serum was weakly positive for IgG GQ1b and GT1a, and serum IgE was significantly elevated. He was transferred to a rehabilitation center with Medical Research Council grade 3 lower extremity weakness on admission day 65. We would suggest that hyper-IgE-emia may increase the magnitude and rate of neural damage in this case.