Neonatal central diabetes insipidus (DI) is extremely rare and etiology has not been documented extensively. Asphyxia, intraventricular hemorrhage, severe infections, and central nervous system abnormalities have been associated with central DI in neonatal intensive care units (NICU). Desamino-8-D-arginine vasopressin (DDAVP) has been in clinical use for the treatment of central DI. DDAVP preparations are available for intranasal, oral, subcutaneous, and intravenous administration. There is not clear data for the management and used DDAVP form for the central DI in neonates. In this article, we presented eight cases with different etiology of neonatal central DI. Six cases were preterm with intracranial hemorrhage and the other two patients were congenital toxoplasmosis one of which was preterm. All of the cases received oral desmopressin at a dose of 10 mcg/kg/day. And then oral desmopressin dosage was adjusted according to the serum sodium and urine output. All cases were treated successfully with oral DDAVP. Three cases who have intracranial hemorrhage died due to other preterm complication. According to our case series, oral DDAVP is an applicable, safe and effective form of DDAVP.