The Langerhans cell histiocytosis (LCH) corresponds to an abnormal proliferation of dendritic cells, clonal type, which usually involves compromise of skin and mucous membranes, nails, bone, bone marrow, liver, spleen, lymph nodes, lung, lower gastrointestinal tract, endocrine system and the central nervous system. We present three cases of the disease, with oral and craniofacial manifestations, analyzed from the clinical perspective (intra and extra oral exam), imaging (CT scans) and histopathological (specific marker CD1a expression). Two cases were classified as acute disseminated LCH presentation and one as a chronic disease. Patients were treated with chemotherapy timely according to the protocol of the National Child Program of Antineoplastic Drugs.

KEY WORDS: Histiocytosis; Langerhans cell; Hand-Schüller-Christian; Letterer-Siwe; Hashimoto-Pritzker.