Neurofibromatosis type 1 (NF1) is an autosomal dominant hereditary disease of high penetrance and variable expression. Epidemiologic data on craniofacial manifestations are still lacking. Up until now 74 patients with NF1 have been treated at the Department of Oral and Maxillofacial Surgery of the University of Hamburg. Forty-two patients presented periorbital and orbital neurofibromas varying in extension and in the severity of findings of the affected site. Surgical therapy is mainly based on tumour reduction, frequently combined with face-lifting. In our experience neurofibromas of the neck tend to be pseudo-encapsulated, facilitating the preparation of the tumour. On the other hand, identification and preparation of diffuse infiltrating neurofibromas in the trigeminal nerve region are difficult and local recurrence must be expected.