A preterm Low Birth Weight (1.3 kg) child, born with antenatally detected ventriculomegaly and bowel distension developed respiratory distress and hugely distended abdomen within 6 hours of birth. Preliminary clinical and radiological assessment revealed hugely distended proximal bowel with splinting of the diaphragm. Due to severe abdominal distension and suspected ischaemic bowel, emergency laparotomy was done at 7th hours of birth. A rare (1:10000-20,000 LIFE BIRTH) Type 3 Jejunal atresia with Apple peel deformity of distal bowel(anomalously supplied by inferior mesenteric artery trunk) and thick putty-like meconium in the hugely distended Jejunal blind loop was encountered. The bowel was decompressed, the viability of bowel was restored and a Chimney bowel repair was done. The compliance of lung was restored and ventilation could be normalized. The child has been recovering well in NICU and been started on feeds.There is no scar on her abdomen or perineum which is a great cosmetic benefit to the girl child. Fetal volvulous is an extremely rare condition and a challenge to diagnose and manage. Only few cases have been reported in the world literature with instances of fetal death , still births in neglected cases. It’s a complex multidisciplinary approach with state of the art NICU care which helps in survival of potentially morbid and lethal condition.