The last major review of the etiologies of central diabetes insipidus in children was performed a quarter century ago, prior to the development of modern neuroimaging techniques. We retrospectively reviewed the records of children with central diabetes insipidus identified at Children's Hospital of Buffalo from 1979 to 1992. Of the 35 patients identified, 27 were males and 8 were females. Their ages ranged from 3 weeks to 20 years. Nineteen children had brain tumors, 7 had cerebral malformations, 3 had central nervous system infections, 1 had traumatic brain injury, and 5 were considered idiopathic. Patients with brain death were excluded from the review. Thirty-one of 35 patients developed diabetes insipidus in conjunction with other endocrinopathies. Brain tumor and its treatment account for the most common cause. Cranial magnetic resonance imaging has improved the identification of structural lesions and the understanding of the pathophysiology of central diabetes insipidus.