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In Langerhans cell histiocytosis, diabetes insipidus is the most common endocrinologic complication. We experienced a case of Langerhans cell histiocytosis, involving pituitary stalk and lung. The patient was a 43 year old male with complaint of polyuria and polydipsia. The water deprivation test was carried out to confirm the diagnosis of diabetes insipidus. We found multiple small cysts and nodules in HRCT of lung, and diagnosed Langerhans cell histiocytosis by transbronchial lung biopsy, The patient was managed conservatively with DDAVP nasal spray. The polyuria,polydipsia was relieved completely. After that, we follow up and observe closely the patients lung and pituitary lesion.