RESUMEN
INTRODUCTION: Aggressive fibromatosis is a rare histologically benign fibrous tumor with a potential for locoregional aggression. Treatment is not well defined and several therapeutic approaches have been proposed. Surgical treatment is the reference, chemotherapy, radiotherapy and homonotherapy being proposed as complementary treatment or for inoperable tumors. CASE REPORT: A three-year-old patient underwent surgery for removal of a mandibular tumor. Pathology reported aggressive fibromatosis. The patient was given conservative treatment and was free of recurrence nine years after resection. DISCUSSION: Conservative surgery for aggressive mandibular fibromatosis appears to be preferable to radical mutilating surgery which would have a major impact on facial growth in children.