Langerhans Cell Histiocytosis is a rare and protean disease of unknown etiology, clinically characterized by single or multiple osteolytic and/or soft tissue lesions. Oral involvement may predate systemic manifestations or mimic infectious/inflammatory disorders. In the present series, oral involvement characterized eight cases of pediatric Langerhans Cell Histiocytosis and oral symptoms represented a very early sign of this disease in four patients. A careful clinical investigation and a proper histologic analysis were required for achieving a definite diagnosis and for confirming clinical suspicion of disease relapse. Immunohistochemistry, that nowadays represents a reliable and accessible ancillary histological technique, allowed the definite diagnosis in all cases of our series.