The infant, a girl weighing 2.83 kg (6.24 lb), was the second child of Papuan parents. Delivery had been by cesarean section at term for a major degree of placenta praevia, and no hydramnios had been noted prior to delivery. Physical examination of the child showed a slight anal dimple but no anal orifice. No other abnormality was detected on external examination. A straight x-ray film of the abdomen with the infant inverted showed a large gap from the anal dimple to gas in the colon. Thirty-six hours after birth an intravenous infusion was commenced via the umbilical vein, and the perineum was explored under a general anesthetic. Perineal exploration failed to reach the rectum so a laparotomy through a transverse lower abdominal incision was performed. The ileum was grossly distended and this terminated in a blind end on which a normal appendix was seen. From this point onwards, there was a complete absence of colon and rectum. Although distended and blue, the terminal ileocecal region was thought to be viable. The ap¬ pendix was removed and a terminal cecostomy performed in the right iliac fossa. The infant died on the second post¬ operative day from a peritonitis following perforation of the ileum.Postmortem examination confirmed the antemortem finding of an absent colon and rectum. The intestine was found to have ruptured 12 cm from the cecostomy and had produced a meconium peritonitis. In addition, there was a complete absence of the left kidney and ureter and of the left tube and ovary. The right kidney and ureter were normal, and the uterus ap¬ peared to be unicornate with a normal right horn, tube, and ovary. The left adrenal was considerably larger than the right. All other organs were normal. There were two umbilical arteries present. His¬ tological examination of the iliocecal re¬ gion showed a change in the lining epithelium from small bowel to cecal pat¬ tern approximately 2 cm from the termi¬ nation. The colon was completely absent from the early ascending colon onwards.There was no rectum. There was no sign of any fibrous band or residual structure that might have represented a hypoplastic colon.