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The acute peripheral neuropathy as one of hypereosinophilic syndrome is known to be a rare disorder. The authors experienced a dramatic case with acute peripheral neuropathy, hypereosinophilia in peripheral blood, and the positive anti-GM1 antibodies. The serum protein electrophoresis showed a diffusely increased gamma-globulin region and the polyclonal gammopathy was found by the immunoelectropheresis. There was no evidence of inflammatory myopathy in vastus lateralis muscle. The sural nerve biopsy was compatible with vascular neuropathy, as there were a few myelin digestion chambers, mild perineuronal fibrosis, and perivascular lymphoplasmocytic infiltration with focal organizing thrombosis. The clinical response to prednisone therapy was excellent.