A girl with chronic idiopathic neutropenia who developed gingival enlargement at seven years of age is presented. Intraoral examination revealed generalized gingival inflammation with a tendency to bleeding and inflammatory gingival enlargement localized to the anterior region. A considerable amount of bacterial plaque was noted on the teeth. There were also 4-5 mm pocket depths around the first molars. Radiographic examination also indicated the presence of incipient bone loss around the first molars in both jaws. The patient, who was diagnosed as localized prepubertal periodontitis with generalized gingival inflammation and anterior gingival enlargement, accentuates the importance of evaluation of periodontal status in patients with chronic idiopathic neutropenia, to avoid the destruction of supporting structures of the dentition.