RESUMO
Desmoplastic fibromas of bone are extremely rare, slow growing, locally invasive, benign primary bone tumours, bearing close resemblance to the extra-abdominal desmoid tumours of soft tissue. They typically occur in patients around 30 years of age, and most commonly affect the mandible, pelvis and meta-diaphyseal region of long bones. En bloc or wide resection has typically been the treatment of choice to avoid recurrence, however, recent reports support curettage with bone grafting and adjuvant therapy to minimise functional loss. We report a 9-year-old child with a desmoplastic fibroma of right radius. This is an unusual age group for this bone tumor. The tumor was managed with en bloc resection and reconstruction with a non-vascularised fibula autograft. The patient had good functional outcome and no recurrence at 1-year follow-up. Level of Evidence: Level V (Therapeutic).