A rare case of neurofibroma in a two-year-old boy is reported, with details of light microscopy and immunohistochemistry. Microscopically, the tumor was composed of large spindle cells and small round cells. Immunohistochemically, moderative positivity for S-100, S-100 alpha and NSE was seen in the large spindle cells, but only weak positivity was evident in the small round cells. The large spindle cells were thought to be more differentiated than the small ones, because S-100 beta, a marker of well-differentiated nerve tumors, was identified only in the spindle cells. The large spindle cells may be derived from Schwann cells, and the small round cells from the perineurium cells. The literature on neurofibroma in children was reviewed and discussed.