Our aim is to compare the different methods of treatment available for Langerhans cell histiocytosis (LCH) in the oral and maxillo-facial region. A classification based on clinical and immunohistochemical data is proposed and a grading for the severity of the disease is proposed.Records and clinical data of 12 patients were evaluated retrospectively. The patients' ages ranged from 20 months to 47 years. Nine had manifestations in the maxillo-facial region, of which six had a single oral lesion only, with the three remaining patients having multiple lesions in this region. Three patients had lesions in the upper thorax in addition to their oral lesions. Eleven patients were treated surgically with one having been treated previously with steroid-therapy. One patient had already been treated with chemotherapy. The longest follow-up was 16 years. Immunohistochemical stains for antigen-CD-1a, antigen-S-100 and antigen-Ki-67 were performed. A proposal for staging the lesions in the oro-maxillo-facial region was made. The intensity of the antigen-Ki-67 stains was evaluated.Eleven patients exhibited no signs of recurrence whilst only one patient had a recurrence implying that surgical treatment was very effective in eradication and cure of the disorder. It is felt that the number of antigen-Ki-67 positive cells is related to the activity of the disease.Langerhans cell histiocytosis should be treated surgically. Only in very severe cases should surgical treatment be complemented with either radio-therapy or chemotherapy. Especially in disseminated cases, chemotherapy would appear to improve the outcome. The antigen Ki-67 as proliferation marker is suggested as a grading parameter of this disease.