Langerhans Cell Histiocytosis (LCH) is an uncommon disorder marked by the proliferation of specialized dendritic cells that can infiltrate various organs. Characterized by its diverse clinical presentations, LCH most frequently appears as solitary eosinophilic granulomas in bone, primarily affecting children. Despite its rarity, with an incidence ranging from 2 to 5 cases per million annually, LCH can present at any age and often mimics other conditions, making accurate diagnosis challenging. Clinical manifestations may vary widely, from localized bone lesions to systemic involvement.
In pediatric patients, LCH can present as unifocal bone lesions, often leading to misdiagnosis if not thoroughly evaluated. A case in point is an 18-month-old female who presented with a progressive swelling in the left cheek, initially suspected to be a dental infection.
Subsequent imaging and biopsy confirmed the diagnosis of unifocal LCH of the mandible. This case highlights the importance of considering LCH in differential diagnosis, especially when dealing with atypical bone lesions in children

Keywords:Langerhans Cell Histiocytosis; Eosinophilic Granuloma; Mandibular Lesion; Pediatric Bone Disease; Ameloblastoma.