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Langerhans cell histiocytosis (LCH) is a disease characterized by the proliferation of Langerhans cells in one or more organs. The cause of the disease is not very clear, it can occur at any age, but it is more common in infants, there is currently no uniform treatment for LCH. Here we report a case of "polyuria, thirsty and polydipsia" as the first manifestation of an adult female patient. It gradually developed into multiple lesions and multiple systems involving pituitary, skull, left clavicle, spine and left femoral head and so on. LCH was confirmed in this patient by pathological section which was langerin (+ +). The adult multifocal LCH cases admitted as central diabetes insipidus (CDI) were very rare in our department, for the patients with pituitary stalk thickening, bone tissue and multiple organ involvement, the possibility of LCH should be considered and histopathological biopsy should be carried out, which is very important for the diagnosis and treatment.

Multifocal adult Langerhans cell histiocytosis with diabetes insipidus as the initial symptom: a case report and literature review