Odontogenic keratocysts make up 4%–12% of all odontogenic cysts. Most cysts are
sporadic but sometimes they arise in the context of basal cell nevus syndrome
(Gorlin syndrome). Most odontogenic keratocysts arise in the posterior region of
the mandible, but they can occur anywhere in the jaw. In rare instances, they
are located peripherally in the gingiva. Even more rare, they are found in the
soft tissues of the mouth. There have been a few case reports and small case
series of such peripheral odontogenic keratocysts. Some controversy exists as to
whether these truly represent a peripheral counterpart of the intraosseous
odontogenic keratocysts and if their origin is at all odontogenic. We hereby
present two cases of peripheral odontogenic keratocysts, both being located in
the soft tissue of the buccal mucosa, and review the literature on peripheral
odontogenic keratocysts.