Rare Association of Type 1 Omphalocele With Ileocecal Atresia and Congenital Appendiceal Agenesis: A Case Report.

Omphalocele is a relatively common congenital abdominal wall defect encountered in the neonatal period, whereas intestinal atresia and appendiceal developmental anomalies are much rarer entities. The association of these malformations represents an exceptionally rare clinical condition, scarcely reported in the literature. We report a case of a female neonate presenting with a type 1 omphalocele associated with ileocecal atresia and congenital appendiceal agenesis. Surgical management included reduction of the herniated viscera, primary ileocolic anastomosis, omphalocele repair, and a prophylactic Ladd procedure. The postoperative course was uneventful, with early return of bowel function and progressive advancement of enteral feeding. This case is reported due to the extreme rarity of this triad of congenital anomalies and to highlight key diagnostic and therapeutic considerations in neonatal surgical management. It also emphasizes the importance of early, tailored surgical intervention in achieving excellent functional outcomes.