We present a case of subcutaneous plexiform schwannoma, a rare benign perip
heral nerve sheath tumour characterized by a multinodular and plexiform gro
wth pattern. A review of the literature was performed to identify the relat
ionship between plexiform schwannoma and neurofibromatosis types 1 and 2, a
nd schwannomatosis.
It is also important to distinguish plexiform schwannoma from plexiform neu
rofibroma, a particular type 1 neurofibromatosis lesion, because of the pro
pensity of the latter for malignant degeneration.