Schwannomas, arising from nerve-sheath Schwann cells, rarely occur in the lip area. A Medline search in English literature from 1966 to 2002 revealed only 6 documented cases of schwannomas of the lip. We report the unusual case of a 22-year-old female patient who developed a schwannoma of the upper lip. Histopathological examination and immunohistochemical staining studies were performed, with the relevant literature for this clinical rarity also reviewed. Complete enucleation of the schwannoma usually achieves cure. Although very rare, schwannoma should be taken into consideration in the differential diagnosis of any upper lip mass.