Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology that predominantly affects children. It can involve multiple organs throughout the body, with skeletal involvement, particularly in the craniofacial bones, being the most common. The clinical manifestations of LCH are diverse, necessitating differentiation from various other diseases, and definitive diagnosis relies on pathologic examination. The authors retrospectively reviewed the data of 9 pediatric patients with mandibular LCH treated at their institution. The authors' study summarized the disease characteristics, differential diagnosis, treatment, and prognosis of mandibular LCH in children, along with a literature review.