Abstract
Langerhans cell histiocytosis (LCH) is a rare reactive and proliferative disease of histiocytes. The disease occurs predominantly in children and rarely in adults. This disease of unknown etiology exhibits extreme clinical heterogeneity. Even though LCH manifests initially in the oral cavity in most of the cases, owing to the relative rarity of the condition, it remains a disease in which the diagnosis is often delayed, missed or misdiagnosed. This is a case of LCH in a child which presented with swelling in the mandibular region.