We present a case of subcutaneous plexiform schwannoma, a rare benign peripheral nerve sheath tumour characterized by a multinodular and plexiform growth pattern. A review of the literature was performed to identify the relationship between plexiform schwannoma and neurofibromatosis types 1 and 2, and schwannomatosis.
It is also important to distinguish plexiform schwannoma from plexiform neurofibroma, a particular type 1 neurofibromatosis lesion, because of the propensity of the latter for malignant degeneration.