Background: Intestinal obstruction is the most common surgical emergency in the newborn period. It is a life threatening condition with high mortality especially in developing countries. Neonates with intestinal obstruction require specialized care and facilities for survival.rnObjective: We aim to study the etiology, presentation, outcome, and factors associated with mortality in the setting of Tertiary university hospital at Addis Ababa, Ethiopia.rnMethod: Records of all cases of NIO managed at TASH, NICU during the period of January 1, 2010 to December 31, 2013 were retrospectively reviewed and all the possible factors that may result in mortality were analyzed.rnResults: Fifty-one neonates with intestinal obstruction were managed, representing 0.0058% of the neonatal admissions during the study period. Forty-one (80.4%) were males, 9(17.6%) were females (M: F=4.55:1), while one newborn had ambiguous genitalia. All patients presented with in the first week of life. Anorectal malformation constituted 56.9% of the causes of NIO, while intestinal atresias including duodenal, jejunal, ileal atresias accounted for 13.7% of cases. Other causes included Hirschprungâ€™s disease, intestinal malrotation, meconium plug, congenital band, paralytic ileus, blind sigmoid colon, and spontaneous gastric perforation. There were 10 deaths, with a mortality rate of 19.6%. Perioperative sepsis was significant determinant of mortality.rnConclusion: Early presentation, prevention and prompt management of sepsis is needed to decrease the high mortality seen in this study. There is a need for improved facilities including Intensive Care Units for pre- and postoperative management of neonatal intestinal obstruction.rnKey words: Intestinal obstruction, neonates, anorectal malformation, intestinal atresia