Odontogenic keratocyst displaying foci of cartilaginous metaplasia in the connective tissue wall has been previously reported but is considered rare. We report a case of a 66-year-old man with an odontogenic keratocyst of the mandible. Benign-appearing metaplastic cartilage was observed in the wall of this cyst, as well as in the recurrence. A benign clinical course is documented, and a review of the literature detailing 6 previously reported cases is presented.