Abstract. Desmoplastic fibroma, alternatively known as aggressive fibromatosis or desmoid tumors, occurs in the form of benign locally aggressive tumors that possess a high rate of recurrence. The forearm bones are rarely involved. The current study presents a case of desmoplastic fibroma in the distal forearm of a 23-year-old man. The tumor was widely resected, and the bone defect was reconstructed using an autologous vascularized fibular graft during the resection procedure. The patient experienced recurrence three times and underwent four resections during the subsequent 3 years following the initial resection. After 10 years of follow-up, the patient's functional recovery remains positive. Despite the implication that surgical resection may be involved in the development of aggressive fibromatosis, surgical wide local excision and functional reconstruction were recommended for the treatment of the present patient.