We report a case of chronic relapsing idiopathic polyneuropathy developing six weeks after the onset of extraocular palsy. As in the other four published cases, the patient failed to respond to steroids and also in this case to plasma exchange, thus confirming the poor prognosis. The electrophysiological features and poor response to plasma exchange are at variance with a demyelinating pathogenesis and suggest classification of this form as a distinct clinical variant.