IntroductionLangerhans cell histiocytosis (LCH) is a rare hematological disorder characterized by intense and abnormal proliferation of bone marrow-derived immature myeloid dendritic cells; Langerhans cells (LCs) in the skin, bone, lymph nodes, and other organs.1Although, due to its low occurrence, limited information on the epidemiology of LCH is available, an estimated 5 to 9 cases per million in children < 15 years of age, and 1 case per million in patients > 15 years of age have been reported.2Depending on the different clinical manifestations, LCH had been classified conventionally by the International Histiocyte Society into three different types: Type I - Eosinophilic granuloma (chronic focal LCH), Type II - Hand-Schüller-Christian disease (chronic disseminated LCH) and Type III - Letterer-Siwe disease (acute disseminated LCH).3 However, because clinical findings are widely manifested, the disease has also been classified into unifocal and multifocal forms; depending on the extent of its spread throughout the body. The unifocal form presents only one destructive lesion in an organ, affecting bones or skin (80% of cases), liver, spleen, brain, and lymph nodes more frequently3 The disease manifests itself in a variety of ways, from isolated disease that resolves on its own to life-threatening multisystem disease, with a 20% mortality rate.4The involvement of head and neck region is quite common in LCH, particularly the bones of skull and jaws. The oral soft tissue lesions are also common and are mostly seen on the hard palate and gingiva. Since the symptoms of LCH may first appear in the oral cavity before occurring elsewhere in the body, a thorough knowledge of the clinical presentation of the disease is important.1Awareness among clinicians regarding the clinical, histological, and immune-histopathological features of the disease will help in early diagnosis and treatment for better prognosis. Thus, this report aims to present a case of Langerhans cell histiocytosis with the oral and dermal manifestations seen in a pediatric patient.