Lipofibromatous hamartoma is a rare tumor of the nerve. The median nerve is the most involved nerve. The nerve is diffusely enlarged and normal nerve bundles can not be separated from this tumor. It is frequently associated with enlargement of finger(s) in the median nerve territory. This always opens the discussion in which way macrodactyly is nerve driven. The tumor can also be the cause of (childhood) carpal tunnel syndrome. We report a case with additional arguments for the neural influence on growth.