Abstract： A 42-year-old male presented with alopecia areata and swelling of the scalp for over two years.Dermatological examination revealed a soft,well-marginated,skin-colored and elevated mass sized 10 cm × 8 cm in the left scalp with overlying hair loss.The mass had a ‘bag of worms' feel on palpation.Histopathological examination demonstrated an ill-defined,infiltrative mass occupying the entire dermis and part of the subcutaneous tissue,without the involvement of skin appendages.The tumor was composed mainly of short spindle cells,which were surrounded by loose myxoid matrix with wire-like collagen fibers and scattered mast cells.Meissner bodies were easily seen.Fifty percent of the tumor cells expressed S-100 protein.A diagnosis of diffuse neurofibroma was made.The patient refused operative treatment because of large involvement of the head,and follow-up was under way.