[Desmoid infantile mandibular fibromatosis]. / Fibromatose desmoïde infantile de la mandibule.

INTRODUCTION: A desmoid tumor, or aggressive fibromatosis, is a benign fibrous tumor with a high potential for locoregional extension. This tumor is very rarely located in the mandible. OBSERVATION: A 2-year-old boy presented with an extensive mandibular desmoid tumor. The diagnosis was proved on histological examination. Two years after surgery, there was no recurrence. DISCUSSION: Aggressive fibromatosis is rarely located in the mandible. The differential diagnosis with malignant tumors is difficult. Surgery is the first-line treatment. However, alternative therapies should be considered, especially in children, to avoid mutilating operations.