OBJECTIVE To determine the etiologies and associated endocrine disorders in children with central diabetes insipidus (DI). METHODS The authors retrospectively reviewed the medical records of children with central DI, who were admitted at department of Pediatrics, King Chulalongkorn Memorial Hospital, between 2000 and 2004. Aims of this study were to identify the etiology of central DI in children and also described the anterior pituitary hormone insufficiencies which may occur. RESULTS Of the total 51 patients, 27 patients were males and 24 were females. Intracranial tumors produced DI in 36 children (70.6%), but 17 of these 36 children (47.22%) had DI before surgical removal of the tumors. Fifteen patients (29.4%) had DI from non-tumor causes, which include idiopathic in 2 patients (13.5%), terminal events in 4 patients (26.8%), central nervous system (CNS) infection in 5 patients (33.3%), CNS anomalies in 2 patients (13.5%), Kabuki syndrome in 1 patient (6.6%), head injury in 1 patient (6.6%). Anterior pituitary function was evaluated in all tumor group and 8 patients of non-tumor group. In intracranial tumor group, growth hormone deficiency (GHD) was documented in 14 from 22 patients (63.6%), secondary adrenal insufficiency in 13 from 20 patients (65%), central hypothyroid in 27 from 36 patients (75%), hyperprolactinemia in 5 from 8 patients (62.5%). CONCLUSIONS The most common etiology of central DI is intracranial tumor, and at least 50% of them have clinical features suggesting central DI before surgery. More than 60% have associated anterior pituitary hormone insufficiency.