The occurrence of primary segmental volvulus (PSV) is very rare. The main symptoms are abdominal distension and vomiting ol bile. ln gur case, a 1-day-old neonate weighing 1,960 grams presented with repeated vomiting and lailure to defecate. Due to progressive abdominal distension, failure to defecate and bile reflux into the Gtube, a laparotomy was performed on day 3 of life. The stapled segment and the abnormally dilated bowel segment were resected. No other obstruction was found by probing the opened bowel segments in the oral and aboral directions. The two bowel segments were loined by an end-to-end anastomosis with pieservation of the cecum. To relieve the anastomosis, an appendicostomy was created and a feeding tube was placed into the ileo-jejunal segment to relieve the strain. After surgery the bile reflux and abdominal distention gradually resolved. Neonatal small bowel segmental volvulus is a very rare entity but should be considered in the differential diagnosis because of its high morbidity.  Keywords: primary segmental volvulus, surgery, a 'bird's beak' pattern