| Summary: | Desmoplastic fibroma (DF) is a rare benign intra-osseous tumor with provincially aggressive nature. The tumor constitutes <1% of bone tumors, and mandibular involvement is common with a reported frequency of approximately 40% of the various bony sites. It may progress to bone destruction and displays a tendency to invade the soft-tissues if untreated. Inadequate treatment accounts for the high propensity for local recurrence. We report a case in a 6-year-old female child with a swelling over the left body of the mandible, an expansile lytic lesion with the destruction of lingual and buccal cortex noted on computed tomography (CT) scan. The child underwent left hemimandibulectomy and reconstruction of the sick jawbone. Recognition of this entity is important because it may histologically and radiographically resemble benign fibrous lesions. The non-specific clinical appearance of the DF implies an extensive imaging study before in order to establish a proper therapeutic approach. We hope that our case report will shed new light on this special entity.
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