[Desmoid infantile mandibular fibromatosis]

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.

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.

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.