Nervous lipofibromatous hamartoma is a rare tumor-like condition involving the peripheral nerves, whereby the epineurium and perineurium are enlarged and distorted by excess of fatty and fibrous tissues that infiltrate between and around nerve boundaries. The median nerve is much more likely to develop a hamartoma than other nerves with a predilection for the carpal tunnel. We present a case of carpal tunnel syndrome in an adult caused by fibrolipomatous hamartoma of the median nerve, successfully removed by excision of the fibrolipomatous tissue and decompression.