A case of lipofibromatous hamartoma of the median nerve in an adult is described in this article. A 33-year-old male presented with tingling, numbness and swelling in the palm of the left non dominant hand that had been present for a few months. Examination revealed that there was fullness in the volar aspect of the wrist and in the inter-thenar area. Another mass was present at the base of the index finger, which appeared to be involving subcutaneous tissues. The clinical diagnosis was carpal tunnel syndrome due to a space occupying tumor mass in the carpal tunnel. On exploration of the carpal tunnel, a large median nerve was seen 4 cm proximal to the wrist crease line and extending distally until it divided into its branches. Another mass was present at the base of the index finger, which was adherent to the skin. The radial digital nerve of the index finger was normal in size. For the enlarged nerve, an epineurotomy was performed and a biopsy was taken. Another biopsy was taken from the distal mass. His postoperative period was uneventful. In July 2004, at the end of 5 years, the patient had no symptoms and the size of the tumor had not increased.