Objectives: The fibromatosis that occure in the head and neck, including those that involve oral and paraoral structures, are considered under the subheading of extra-abdominal fibromatosis. Infantile fibromatosis usually arises as a solitary mass in skeletal muscle or in adjacent fascia, aponeurosis, or periosteum. Head and neck lesions of IF are primarily found in the tongue or in the region of the mandible or mastoid process. In this report, a seldom case, infantile fibromatosis, with prepubertal periodontitis have presented. Methods: 5-year-old girl has prompted to seek treatment in 2000 because of generalized gingival hyperplasia and gingival bleeding during nutrition and brushing. In the clinical examination, generalized gingival hyperplasia, proliferative and hemorrhagic gingiva and deep periodontal pockets, and in the radiographic examination generalized alveolar bone loss and vertical defects around deciduous have observed. The periodontal examination when evaluated with the radiographs has defined the case as prepubertal periodontitis. The consultation with the pediatric physicians has revealed any systemic disease. Results:She received oral prophylaxis, scaling and root planning, the teeth with poor prognosis have extracted and the gingival contours have corrected with the gingivectomia procedure. The biopsies taken during the operations have been diagnosed as infantile fibromatosis after the histopathological evaluation. The patient has seen in every 3 months during the 4 year follow up period. The periodontal surgical therapy has repeated in the case of recurrence. The interrelationship of the teeth and occlusion of the remaining teeth have been corrected by the orthodontic therapy. Conclusions: The therapy of infantile fibromatosis has been recommended complete excision of the lesions. The close follow up is necessary because of the recurrence trend of the lesions.

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