Central diabetes insipidus (DI), resulting from disorders that act on the site involved in the secretion of antidiuretic hormone (ADH), leads to polyuria, polydipsia, and hypernatremia. Langerhans cell histiocytosis (LCH) is one of the causes. LCH, a rare disorder in which granulomatous deposits occur at multiple sites within the body, could involve the hypothalamo-pituitary axis and DI would be a common manifestation. We report the case of a 52-year-old woman, with initial presentation of general weakness, polyuria, and polydipsia, who had hypernatremia (serum sodium level: 173 mEq/L) and central DI was diagnosed after a water deprivation test. Brain magnetic resonance imaging (MRI) revealed a mass lesion in the hypothalamus. The pathological report after surgical intervention revealed LCH. Here, LCH and her clinical course are briefly discussed.