Aggressive fibromatosis is a rare tumor of the jaws. Its occurrence in the maxilla is even more exceptional. The case of a 14-year-old girl with a peripheral fibromatosis of the palatal process of the maxilla is described. The lesion manifested as a painless swelling. On initial excision, a diagnosis of fibroma was made. After recurrence and reevaluation of the microscopic slides, a final diagnosis of aggressive fibromatosis was established. The features of this case and 12 similar lesions of the maxilla previously described in the literature are analyzed and discussed.