Introduction: VACTERL/VATER association is conventionally diagnosed when three or more ofthe following congenital anomalies are present: vertebral defects, anal atresia, cardiac abnormalities, Oesophageal defects, renal anomalies, and limb defects. Bifid rib is a rare association of VACTERL anomaly . Here we present a rare case of VACTERL anomaly where Oesophageal Atresia(OA) needed complicated multistage surgical interventions. Case presentation: A male neonate was delivered at 36 weeks of gestation via emergency Caesarean section for polyhydramnios, with a birth weight of 2440 g. Postnatal evaluation revealed a butterfly vertebra, a bifid rib, a moderate-sized ostium secundum atrial septaldefect, OA, and a left-sided duplex kidney . The proximal esophageal pouch was markedly dilated at the level of the azygos vein, while the distal segment appeared as a small protrusion at the diaphragmatic hiatus. On day 3 of life, he underwent right posterolateral thoracotomy and primary OA repair. Thepostoperative course was complicated by an anastomotic leak. Subsequently , on days 19, 26, and 54 of life, he required repair of the anastomotic site with feeding jejunostomy , laparotomy with gastrostomy creation, and central oesophagostomy formation, respectively . His hospitalcourse was further complicated by chylothorax and secondary lactose intolerance which were managed accordingly . Despite the prolonged and complex clinical course, the infant was eventually stabilized with weight gain and discharged. Conclusion: VACTERL association may present with rare phenotypic variations. Long-gapesophageal atresia is surgically challenging, and its management remains controversial. The initial attempt to do primary repair was unsuccessful, ultimately necessitating alternative staged surgical interventions.