Proteus syndrome is a rare congenital disorder comprised of subcutaneo
us and internal hamartomas, pigmented skin nevi, skull exostoses, hemi
hypertrophy, and macrodactyly of the hands and feet. A 5-year-old girl
diagnosed with Proteus syndrome presented with distal median compress
ion neuropathy with the primary complaint of severe pain involving the
left hand. Surgical exploration of the hand revealed a lipofibromatou
s hamartoma of the median nerve. The transverse carpal ligament was re
leased and epineurectomy of the median nerve was performed. The patien
t remains symptom free at the 9-month follow-up. This report is the fi
rst description of a hamartoma directly involving a peripheral nerve i
n Proteus syndrome. Decompression of the nerve with the removal of the
fibrofatty neural sheath resulted in the resolution of the symptoms i
n this patient. The surgeon should consider this approach as a potenti
al first line of treatment before a more radical resection of the nerv
e is contemplated.