Segmental neurofibromatosis.

ABSTRACT

Segmental neurofibromatosis is a rare disorder, characterized by neurofibromas or cafι-au-lait macules limited to one region of the body. Its occurrence on the face is extremely rare and only few cases of segmental neurofibromatosis over the face have been described so far. We present a case of segmental neurofibromatosis involving the buccal mucosa, tongue, cheek, ear, and neck on the right side of the face.