Differential diagnosis of Langerhans Cell Histiocytosis (LCH) in the jaw is essential for early treatment including systemic therapy. Records of 17 patients (6 men and 11 women; mean age, 14 years) with histologically confirmed LCH were reviewed. All the lesions occurred in the mandible. Most of the cases (n=12) were intraosseous type LCH, only 5 patients had alveolar type LCH. Patients complained of facial swelling and pain most likely. In the 14 patients who underwent CT and/or MR imaging, all LCH lesions were osteolytic, with a mean size of 23 mm. LCH presented as expansile lesions with periosteal new bone formation, perilesional sclerosis, fluid attenuation/signal within the lesion, and inflammatory changes in adjacent soft tissues on CT/MR images. Considering the major symptoms of LCH were swelling and pain, the differential diagnosis of LCH from osteomyelitis might be more difficult. The differential diagnosis for osteolytic lesions of the jaw with surrounding inflammatory changes should include LCH, especially in young patients.