ABSTRACT

Plexiform neurofibromatosis is a rare nerve sheath tumor originating from schwann cells.

Although histologically benign, it can cause cosmetic and functional deformity. It may be solitary or can accompany other signs of NF. The rate of malignant transformation in this lesion is 2-5%. In this article, a 23 year old woman with painful, red brown pigmented plexiform neurofibroma over the giant congenital hairy nevus in the right thoracic region was presented. In this presentation, accompanying other skin manifestations of plexiform neurofibromatosis, deformities and rate of malignancy in the literature was reviewed. (Turk J Dermatol 2011; 5: 56-60)