A 2-day-old female neonate with the clinical picture of proximal small bowel atresia, on exploration, turned out to have intestinal atresia of a rare variety, i.e., a near-total jejuno-ileal atresia. The baby had total small bowel length of less than 10 cm. She survived for 3 months on enteral feeding after end-to-back duodeno-ileal anastomosis and thereafter succumbed to septicemia. The case is presented for it's extreme rarity and consideration of this extreme form of small bowel atresia as an offshoot of the existing classifications of jejuno-ileal atresia since it has dismal prognosis and presents as a management challenge even today.