Skeletal desmoplastic fibroma is an intraosseous neoplasm that is recognized as a very scare benign tumor. It has a propensity for locally aggressive behavior and local recurrence. The aim of this article is to report a case of skeletal desmoplastic fibroma in right mandible of a 4-year-old boy. The patient was found to have a large skeletal desmoplastic fibroma in right mandible, which was resected by surgical intervention. The defect was successfully restored with a titanium plate. In the report, the etiopathogenisis, pathological, radiographic features, clinical diagnosis, therapy and prognosis of skeletal desmoplastic fibroma were diccussed.
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