Colloid milium (CM) is a rare condition characterized by the presence of multiple dome-shaped amber- or flesh-colored papules developing on sun-exposed skin. It is a degenerative condition linked to excessive sun exposure and possibly exposure to petroleum products and hydroquinone. The origin of the colloid deposition in the dermis is uncertain, although it is thought to be due to degeneration of elastic fibers. The condition is rare, with only 100 case reports documented in the world literature. The most commonly involved sites are the face, periorbital region, backs of the hands, back and sides of the neck, and ears. Involvement of the oral cavity is extremely rare. The purpose of this article is to report an extremely rare first case of CM of the oral cavity and to discuss the clinical, pathologic and therapeutic spectra of CM.