Abstract: Objective To report the diagnosis and treatment of central diabetes insipidusdue caused by primary empty sella syndrome in one case, and review the related literatures. Methods A 42-year-old female patient presented with microsomia, oligomenorrhea admitted to Chinese PLA General Hospital on Feb 29, 2016. She delivered a daughter at age of 24 after artificial cycle treatment, and menopause occurred when she was 38 years of age. She complained of polydipsia and hyperdiuresis, more than 1 year on admission. Pituitary MRI showed empty sella turcica and absence of normal short T1 signal of posterior pituitary. which was consistant with the changes of central diabetes insipidus. Pituitary function test suggested that anterior pituitary (growth hormone axis, ACTH-cortisol axis, gonadal axis) and posterior pituitary function were damaged. Primary empty sella syndrome, and pituitary dysfunction were diagnosed. Results Oral desmopressin acetate treatment was effective on this patient, and symptoms including polydipsia and hyperdiuresis were relieved. No hormones or growth hormone replacement therapy was performed to this patient because of no further fertility requirement. Conclusion Hypopituitarism due to primary empty sella syndrome is generally characterized by anterior pituitary dysfunction. However, posterior pituitary dysfunction was found in some patients, causing central diabetes insipidus. Comprehensive pituitary function assessment should be made for these patients, especially for the patients with polydipsia and polyuria symptoms to avoid missed diagnosis of central diabetes insipidus.