Schwannoma is a benign tumor arising from nerve-sheath Schwann cells. When it affects the mouth, the lips are rarely involved. A MEDLINE search in the English literature from 1969 to 2009 revealed only 17 documented cases of schwannomas of the lip. This report describes the unusual case of a 52-year-old man who developed a schwannoma of the lower lip. Histopathologic examination and immunohistochemical staining studies were performed. Although rare, schwannoma should be considered in the differential diagnosis of any nodule or mass in the oral mucosa. Keywords: CD-34 protein, lip, neurilemmoma, neurinoma, S-100 protein, schwannoma