Typical cases of multiple neurofibromatosis of Recklinghausen are easy to diagnose from a clinical point of view. The mono-symptomatic cases can also be easily diagnosed i f they occur in patients with hereditary backgrounds. However, unusual localization in both types may render the diagnosis difficult. The case to be described in this paper is an example of unusual localization in the head, neck, and oral cavity. From the view point of differential diagnosis, the dentist should be familiar with the disease in its oral manifestation.