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Isolated ascites in a newborn with 'apple peel' jejunal atresia. Isolated fetal ascites was diagnosed at 20 weeks in a primiparous woman with no significant medical history. Progressive fetal ascites worsened after 28 weeks and resulted in fetal hydroceles. Delivery was by caesarian section at 33 weeks, preceded by reduction of fetal ascites under ultrasound guidance. Following delivery, the baby required further reduction of abdominal fluid and endotracheal intubation to provide respiratory support. An extensive set of investigations, including metabolic and genetic screening, was performed; all results were negative. On day two of life, the baby developed bilious aspirates and an abdominal radiograph suggested intestinal obstruction. At laparotomy, an 'apple peel' jejunal atresia, abnormal mesentery with precarious blood supply and a proximal perforation were identified and the perforation 'sewn over'. The postoperative course was unremarkable, with Monogen feeds tolerated three weeks later. The baby continued to thrive at one year, tolerating increasing amount of long-chain fatty acids in diet.