Introduction: Langerhans cells histiocytosis (LCH) is a rare disease characterized by the proliferation of monocytics and macrophagics cells. In children, presents incidence of 2 to 5 cases/million/y. The natural history varies from a benign disorder with spontaneous resolution to progressive fatal disease. About 60% of cases manifest with head and neck symptoms and temporal bone is involved in 4 to 25%.