A case of jejunal atresia with "apple peel" deformity is presented. A 27-year-old woman, gravida 3, para 2, suffered from premature labor pain and vaginal bleeding at 32 weeks of gestation. Prenatal ultrasound examination revealed presence of three areas of cystic dilatation in the fetal abdomen and marked polyhydramnios, indicating a high bowel obstruction. A female baby was delivered by Cesarean section with Apgar scores of 7 and 8 at one and five minutes, respectively. Physical examination showed abdominal distention. Nasogastric tube aspiration got more than 50 ml of blood-tinged fluid. Roentgenography demonstrated a high jejunal obstruction as well as a normally rotated colon. Laparotomy disclosed proximal jejunal atresia with typical "apple peel" appearance of the distal small bowel. An end-to-end anastomosis was performed smoothly after resection of both the proximal and distal small bowels. A reduction tapering antimesenteric jejunoplasty was also done. Partial parenteral nutrition had been administered for 10 days followed by enteric feeding. The postoperative course was uneventful. Her chromosomal analysis revealed a normal karyotype of 46,XX.