Chondromyxoid fibroma is a benign tumor of bone that is characterized by chondroid and myxoid differentiation and by ultrastructural and immunohistochemical evidence of chondral origin. It is rare in the jaws and skull bones, where only about 2% of all cases have been reported. A review of the 20 acceptable gnathic cases in the literature and of the current case revealed both a higher incidence in the mandible (76%) than in the maxilla (24%) and an equal sex distribution. The sites of occurrence in both jaws are compatible with origin from developmental cartilaginous remnants. The controversies regarding malignant transformation and therapeutic approach are addressed.