Background: Malrotation of the intestines is an uncommon pediatric condition that typically presents in the first month of life. Case Presentation: Using the SCARE 2020 Guidelines, we report a case of a 12-year-old female with omphalocele and bilobed liver herniation presenting with chronic intestinal malrotation. A computed tomography of the abdomen and pelvis with IV contrast revealed a peritoneal defect measuring 4 cm in the upper abdomen, containing the gut including pylorus, duodenum while retrospective distension of the stomach was seen up to the pylorus. The open Ladd’s procedure was performed to correct the defect. No complications were noted intra- or 1 month post-operatively. Patients with intestinal malrotation may present with abdominal pain, intestinal obstruction, nausea, or vomiting. Conclusion: The open Ladd’s procedure was deemed safe, feasible, and effective in a resource limited country like Pakistan, in the treatment of young children with intestinal malrotation.

Keywords: Intestinal malrotation, omphalocele, liver herniation, Ladd’s procedure, Pakistan, case report.