• We describe six young patients with insidiously progressive, painless weakness in the distribution of a single major lower extremity nerve. No cause could be found despite extensive evaluation, including surgical exploration. At the time of diagnosis, all patients had weakness and three patients had sensory loss. In all cases, electromyography revealed a chronic axonal mononeuropathy without conduction block or focal conduction slowing. Magnetic resonance, computed tomographic, and ultrasound imaging studies did not identify a region of nerve swelling, mass, or compression. At surgical exploration, the nerve appeared atrophic in two patients, indurated in one patient, and normal in two patients. Biopsy specimens obtained from two abnormal nerves revealed either wallerian degeneration or endoneurial fibrosis. The clinical features of these patients comprise an unusual clinical entity with no known cause or treatment.