Perineuritis unassociated with other significant pathological changes is a rare finding. A patient is described with mild, non-insulin dependent diabetes mellitus who presented with the clinical picture of mononeuritis multiplex, and in whom perineuritis but no occlusive vascular disease was found on sural nerve biopsy. Treatment with prednisone and plasma exchange resulted in some improvement. We conclude that the focal perineuritis, rather than the diabetes, was responsible for the clinical picture mimicking mononeuritis multiplex. In conjunction with previous reports, this suggests that perineuritis may be a treatable neuropathy.