RESUMO
Infantile desmoplastic fibromatoses are benign fibrous tissue tumours, non -metastasizing but locally aggressive and with high likelihood of recurrence. Many cases occur in young children between 18 months and 3 years and commonly present as painless mass of the submandibular region. The maxilla is rarely involved. Through this report and a review of literature, we are going to study clinical, pathological and prognostic characteristics of this affection. We report here a case of an aggressive desmoplastic fibroma arising in a 20-months-old infant originating in the left anterior maxillary wall. Clinical examination of the face revealed a non tender, firm, expansion of the left maxilla of 2.5 cm of diameter. The diagnosis of DF of the maxilla was made on the basis of histological features on biopsy specimen. No other location of the fibromatosis was found. The child had a favourable course. DF is an intraosseous, non metastasizing and locally aggressive fibrous tumour. Although its benignity it could engage vital prognosis since it can extend into vital organs