Neurofibromas are a clinical manifestation of neurofibromatos is type I (NF1). Management of these tumors remains a challenge for the clinician. The goal of the present study is to point out treatment guidelines for these lesions. Eighteen patients diagnosed with NF1 and presenting lesions of the craniomaxillofacial district were included in the study. On the basis of clinical evidence and patient's expectations, only six patients of this group underwent surgery. All patients that had no surgery were included in a follow-up protocol to evaluate progression of disease. Four patients who underwent surgery had good functional/aesthetic results, whereas two patients had incomplete rehabilitation.