Annular pancreas is a rare congenital anomaly with the descending duodenum encircled by a ring of pancreatic tissue, which may cause partial or complete obstruction of the duodenum. In newborn, the symptoms can be those of duodenal stasis resulting from partial intestinal obstruction secondary to some degree of duodenal stenosis. A male newborn weighing 2.0 Kg was born by C-section delivery at 37 weeks' gestation to a 27-year-old mother who had a hydramnios. He was in relatively good condition at birth except regurgitation of saliva and intermittent apnea. A plain film of the abdomen showed the double-bubble of gas filled stomach and proximal duodenum, and upper gastrointestinal series showed a dilated proximal duodenum, with a complete obstruction of the descending duodenum. Intraoperative finding revealed encirclement of the duodenal second portion by pancreatic tissue. Duodenojejunostomy was performed. After the operation, he had developed two serious complications, neonatal septicemia by Enterobacter cloacae on postoperative day 12 and systemic candidiasis on postoperative day 19, and been managed with ventilatory support, antibiotics, and antifungal agents with recovery.