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Background Congenital hernia of umbilical cord is a rare congenital anomaly. Due to its presentation as herniation of small bowel or other viscera into the umbilical cord, it is often misdiagnosed as omphalocele minor. Case presentation We are reporting a rare case of congenital hernia of umbilical cord which presented with rupture of hernia sac and evisceration of small bowel loops with reverse ileal intussusception via a patent vitellointestinal duct. It was managed by first repositioning the gut with stoma at umbilical site as a temporizing measure followed by definitive surgery in the form of resection of patent vitellointestinal duct and end to end ileal anastomosis. Conclusion Congenital hernia of umbilical cord with patent vitellointestinal duct is a rare abdominal wall defect which can present beyond neonatal period with evisceration of gut. A high index of suspicion should be kept by the first attending clinician to avoid a delay in management.

Congenital hernia of umbilical cord with patent vitellointestinal duct along with evisceration of gut—a rare presentation and unusual management