Desmoplastic fibroma is a rare benign tumor of bone that is composed of spindle cells with abundant collagen. This tumor mimics the extra-abdominal desmoid tumor of soft tissue both histologically and biologically. Although desmoplastic fibroma is reported to have a high rate of recurrence, cases of recurrence in the head and neck region have not been reported in the Japanese literature and therefore details are not available. We present here a case of desmoplastic fibroma arising in the mandible of a 24-year-old female. After surgical enucleation, she was diagnosed as having desmoplastic fibroma and we put her under a postoperative course. Three years after the operation, a tumor recurred, so we performed continuous resection of the mandible and immediate reconstruction by using an iliac crest bone graft and titanium plate. As of the time of writing, there has been no evidence of local recurrence for eight years after the operation.
