Langerhans cell histiocytosis is a rare hematologic disease due to multiplication and accumulation of Langerhans cells, which are immature dendritic cells. The clinical presentation is highly variable and can range from isolated, self-healing skin or bone lesions to life-threatening multisystem disease. We report 2 cases confirmed by Histological and immunohistochemical evaluation of temporal bone biopsies. The first case is a 2-year-old child with chronic bilateral otorrhea and polyuria-polydipsia syndrome. Physical examination showed bilateral retroauricular redness and induration with filling all of the right and left external ear canal. Computed tomography showed osseous lysis of the temporal bone bilaterally. Magnetic resonance imaging of the brain showed the presence of pituitary infiltration. The second case is 2-year-old girl with chronic left otorrhea. Clinical examination objectified the presence of redness and induration in the left mastoid with the presence of an inflammatory polyp filling the entire left external auditory canal. Computed tomography revealed a left temporal bone lysis. Both patients were treated with chemotherapy.