نبذة مختصرة : There are numerous birth defects that can affect the gastrointestinal tract, starting from the oesophagus or stomach to the small and large intestines. Intestinal atresias are one of the most common causes of intestinal obstruction in newborns. The most common location is the jejunum, followed by the duodenum and the colon. Congenital absence of the ileum, caecum, and appendix is an extremely rare condition. Here, an exceptional instance of congenital absence involving the distal jejunum, ileum, caecum, and appendix in a male child is presented, who presented with chief complaints of multiple episodes of bilious vomiting after initiating the first feed and non passage of meconium. Upon initial examination, the patient was suspected to have an intestinal obstruction and subsequently underwent an exploratory laparotomy. However, intraoperative findings revealed a grossly dilated proximal jejunal loop, and the next segment of the intestine was the narrow ascending colon. In due course, the baby recovered well and was discharged. Currently, there is no documented occurrence of a comparable case involving a viable neonate in the existing body of scholarly literature. Surgeons need to understand positional variations and congenital anomalies, as well as the procedures needed to identify these abnormalities during surgery.
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