We have described one patient with lipofibromatous hamartoma of the median nerve in the forearm, wrist, and hand and reviewed thirteen reported cases. This uncommon entity, of probable congenital origin, occurs more often in females than in males. It presents as a mass in the volar aspect of the forearm, wrist, or palm, usually during childhood.
Treatment should be transection of the volar carpal ligament with careful removal of any extraneural lipofibromatous material. Although the follow-up of cases reported in the literature has been brief, apparently such treatment produces symptomatic improvement.
Excision of all or part of the mass involving the median nerve certainly produces a significant neurological deficit in the hand and is therefore contraindicat-ed.