A case of fibrolipomatous hamartoma of the median nerve in the palm and digits of the hand in a 14 year old man developed in the right hand is reported. This is rare disease characterized by fatty and fibrous tissue infiltration in themedian nerve of the palm and digits. This case was treated by limited debulking of the redundant tumor tissue and excision of the transverse carpal ligament. This case was performed with relief of the symptom.