A case of keratoacanthoma of the maxillary gingiva in a 12-year-old white boy has been presented. This is only the fourth case of solitary intraoral keratoacanthoma reported in the English-language literature. A review of the literature on solitary and multiple mucosal keratoacanthomas and the incidence of ectopic sebaceous glands has been included. In all mucous membrane locations where keratoacanthomas have arisen, there have been other reported cases of ectopic sebaceous glands in these areas. These findings have led us to consider that intraoral and perhaps all mucous membrane keratoacanthomas arise i, sebaceous glands or their ducts, whether they be normal in their location, as in the buccal mucosa, or ectopic.