Patient EJO, brunette, female, 23 years old, was referred to the Oral and Maxillofacial Surgery Service of the Piracicaba Dental School (FOP/UNICAMP) for removal of a lesion in the lingual region of the lower anterior teeth. The patient had a clinical diagnosis of neurofibromatosis.
Physical examination revealed facial asymmetry and hundreds of skin nodules all over the body, including the face and neck. Café-au-lait spots were present on the trunk and upper limbs.

Intraoral examination revealed malocclusion, diastemas, absent teeth and a neurofibromatous mass in the region of the lower alveolar ridge, lingually to the incisors. The lesion was firm and painless to palpation, of normal mucosal colouring, non-ulcerated, with a sessile base and about 2.5 cm in extension. Due to its size and its relationship with the teeth, it did not allow adequate hygiene of the region, favouring the accumulation of calculus. The tongue also showed an increase in volume, with the presence of a neurofibromatous lesion with diffuse limits.

On radiographic examination, the presence of retained element 23 was observed, as well as right mandibular hypoplasia.

Surgical excision was performed for the neurofibromatous lesion in the lower anterior region. The lesion in the lingual region was not approached surgically due to the fact that it did not present any functional or aesthetic compromise for the patient. The diagnosis of neurofibromatosis was confirmed by anatomopathological analysis.

The patient is now six months post-operative follow-up, with no complaints and no apparent sign of recurrence of the lesion. The patient is currently under periodontal treatment, with significant improvement of the local supporting tissue conditions.