A case of neurofibromatosis, or von Recklinghausen's disease, was presented. The skin manifestations include the development of numerous, variable sized, smooth-surfaced nodules (benign neural tumors) and asymmetric areas of hyperpigmentation called cafe-au-lait spots. Oral manifestations are not always apparent but, when they do occur, include multiple, discrete, nonulcerated mucosal nodules (typically benign neural tumors). Intraosseous tumors (usually neurofibromas) occasionally develop in the mandible as was reported in this patient. Clinicians should be aware of the characteristics of this hereditary disease, especially the possibility of malignant transformation of the neural tumors.