A neurofibroma of the lingual nerve is a rare clinical finding, the most common lesion site of a lingual neurofibroma being the tongue. In most situations, it is difficult to determine the precise nerve origins. Herein, we report a case of lingual nerve neurofibroma that presented as a submandibular mass, mimicking a submandibular gland tumor or solitary lymphadenopathy. Complete surgical excision of such a lesion for histopathologic examination provides a better treatment and final diagnosis. For a patient presenting with neurofibromatosis and a submandibular mass, a neurofibroma of nerve origin should be considered in the differential diagnosis.