The diagnosis Langerhans' cell histiocytosis subsumes different granulomatous lesions of unknown origin. Regarding the literature there are controversies about the disease being a reactive process, a neoplasm or a malignancy. The chronic generalised form compasses the trias exophthalmus, insipid diabetes which is due to a compression of the pituitary stalk, and multiple osteolytic defects of the skull. The latter is accepted to be an obligate sign. The case of a 30 year old male is presented in which histological and immunohistological evidence was made of multiple eosinophilic granulomas of the upper and lower jaw. At the time of making the diagnosis there was an insipid diabetes. The treatment was planned according to suggestions of an interdisciplinary conference and consisted of two six weeks cycles of chemotherapy with vinblastin and prednisone. The aim was to preserve teeth and alveolar process. After the chemotherapy biopsies were taken from the former affected regions which were negative. Despite the chemotherapy an apposition of bone was determined in the molar region of the lower jaw that a prosthetic rehabilitation was initiated. In comparison to similar cases the course of the disease is different and not predictable. It's control has to be implemented by regular radiographic examination and possible biopsies because of the lack of specific tracers. Keywords: Langerhans' cell histiocytosis, eosinophilic granuloma of the jaws, immunohistology, chemotherapy, prognosis, Langerhanszell-Histiozytose, eosinophiles Granulom der Kiefer, Immunohistologie, Chemotherapie, Prognose