A progressive radial neuropathy of unknown etiology despite 1.5T magnetic resonance imaging (MRI) and surgical exploration was identified as an intraneural perineurioma by a localized Tinel's sign, an enlarged radial nerve at the spiral groove by 3.0T MRI, and a fascicular biopsy. The distinction between the initial diagnoses of inflammatory, demyelinating polyneuropathy and perineurioma was made by immunohistochemistry and electron microscopy. A slowly progressing, localized mononeuropathy should include perineurioma in the differential diagnosis.