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ABSTRACT: Objective: To present a case of Langerhans cell histiocytosis (LCH) involving the pituitary and thyroid that was misdiagnosed as idiopathic diabetes insipidus (DI) and Hashimoto thyroiditis.Methods: The history, clinical findings, laboratory and imaging results, management, and follow-up of the case are presented, and the endocrine involvement is discussed.Results: A 50-year-old female with a 13-year history of DI and a 5-year history of progressive goiter with hypothyroidism had been initially diagnosed with idiopathic DI and Hashimoto thyroiditis. She also had a 10-year history of diabetes mellitus (DM). A partial thyroidectomy was conducted when the goiter caused dyspnea, and the postoperative pathological results suggested LCH. After a thorough examination, the diagnosis of LCH was confirmed, and the patient was treated with chemotherapy and radiotherapy. After the treatment, she went into persistent remission.Conclusion: LCH cases mainly presenting as endocrine disorders are very rare, and the diagnosis tends to be delayed or missed due to atypical clinical manifestations. Lesions or functional disorders of the pituitary and thyroid need to be differentially diagnosed against LCH of these organs. Appropriate therapy and long-term follow-up are necessary for the management of endocrine LCH.Abbreviations: CT computed tomography DI diabetes insipidus DM diabetes mellitus LCH Langerhans cell histiocytosis MRI magnetic resonance imaging TSH thyroid-stimulating hormone