We reported a 61-year-old male with chronic, motor, axonal polyneuropathy. Neurological examination revealed severe muscle weakness in the proximal parts of the four limbs. Sensory examination was normal. The cerebrospinal fluid protein was elevated to 74 mg/dl, and the cell count was normal. The serum antibodies to GM1, GD1a, GD1b, and GQ1b were all negative. Electrophysiological studies showed reduced compound muscle action potentials (CMAPs) suggesting axonal neuropathy, and the nerve conduction velocity was only mildly reduced. After treatment with plasmapheresis (PP) by the immunoadsorption method, his symptoms significantly improved in three weeks, and the cerebrospinal fluid protein, and CMAPs also improved. Only a few studies have been reported regarding patients with chronic, motor dominant, axonal polyneuropathy that responded to immunosuppressive therapies or PP. It remains to be determined whether chronic, dominantly motor, axonal polyneuropathy as seen in the present case is a subtype of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) or a primary axonal immune-mediated neuropathy that is different from CIDP. At present we are not able to answ what kind of clinical or laboratory markers other than an elevated cerebrospinal fluid protein level may help to predict a positive response to immunosuppressive therapy or PP.