Objective: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal proliferation of Langerhans cells that affects various organs. Oral involvement may simulate periodontal disease and cause significant diagnostic and management difficulties. Here, we present an interesting LCH case with severe periodontal destruction in a young woman in order to facilitate early recognition of this aggressive disease and successful participation of the general practitioner in the management of such patients. Case Presentation: A 21-year-old woman was referred for evaluation of recurrent episodes of dull pain in the gingiva for the last 9 months, which had not been successfully managed by her general practitioner. Clinical and radiographic examination showed extensive alveolar bone loss. Histopathologic examination revealed diffuse aggregates of Langerhans cells, while a complete work-up did not demonstrate evidence of systemic involvement. A diagnosis of LCH limited to the oral cavity was established. The patient received systemic chemotherapy in combination with appropriate dental care including gingival debridement and tooth immobilization. Following chemotherapy completion, comparative clinical, radiographic, and microscopic evaluation showed complete remission. During an 18-month follow-up period, frequent oral examinations and appropriate dental interventions confirmed the lack of LCH recurrence and guaranteed the stabilization of periodontal tissues. Conclusions: Oral soft and hard tissue involvement may be the only manifestation of LCH. The present case exemplifies the importance of close collaboration between general dentistry and its disciplines (periodontology, restorative dentistry, oral medicine, oral and maxillofacial pathology, and oral radiology), and hematology-oncology for diagnosis, management, treatment monitoring, and decision-making. Schlagwörter: aggressive periodontitis, general practitioner, jaws, Langerhans cell histiocytosis, multidisciplinary approach, osteolytic lesions