
Neurofibromatosis type 1 (NF1, or Von Recklinghausen’s disease) is a genetic disorder transmitted as an autosomal dominant trait with variable expressivities and complete penetrance. It is characterized by a variety of clinical manifestations, including multiple neurofibromas that are associated with a high risk of sarcomatous transformation. This article reports a case of facial plexiform neurofibromatosis with mandibular hypoplasia and reviews various oral soft tissue and hard tissue manifestations of neurofibromatosis I.