Background: Langerhans cell histiocytosis (LCH) is a category of poorly understood disorders characterized by the abnormal growth and accumulation of Langerhans cells (LC) and eosinophils in various organs. The exact cause of LCH remains unknown, but it can result from alteration to the immune system, modifications in genetics, or an infectious process. LCH can be present in localized and systemic forms, affecting different organs depending on the patient's age, disease extent, and systemic involvement. Treatment modalities for LCH could be used individually or in combination, such as surgery, chemotherapy, radiotherapy, or even steroid injections. Also, a few reported cases of the localized subtype showed a spontaneous recovery. Objective: We aimed to delineate the diagnostic approach and treatment modalities for cases with Mandibular (LCH) that might have airway compromise. Subjects and methods: A 4-year-old girl, previously healthy, presented to the Emergency Department (ED) with a history of swelling over the mandible for the last 3 weeks. Results: Results: Symptoms started as toothache that was not interfering with eating or limiting her activity. No swelling or change in color over the jaw were observed. Jaw swelling started to appear, increased in size and interfered with eating. She was diagnosed provisionally as dental abscess formation and received other antibiotics for 5 days, but unfortunately her condition was complicated with bleeding from the swelling and drooling of saliva and yellowish discharge as well so visited our ED. She lost one kilogram during this time. LCH was confirmed with transoral tissue biopsy. Conclusion: Mandibular Lngerhans Cell Histocytosis is one of the differential diagnosis of Osteolytic lesions that need diagnostic approaches and modalities of treatment with paying attention to upper airy way involvement.