Summary

Congenital hernia of the cord, also known as umbilical cord hernia, is an often misdiagnosed and under-reported entity, easily confused with a small omphalocele. It is different from postnatally diagnosed umbilical hernias and is believed to arise from persistent physiological mid-gut herniation. Its incidence is estimated to be 1 in 5000. Unlike an omphalocele, it is considered benign and is not linked with chromosomal anomalies. It has been loosely associated with intestinal anomalies, suggesting the need for a complete fetal anatomical ultrasound evaluation. We present a case of a fetal umbilical cord hernia diagnosed in a 28-year-old woman at 21 weeks gestation. The antenatal and intrapartum courses were uncomplicated. It was misdiagnosed postnatally as a small omphalocele, causing unwarranted anxiety in the parents. Increased awareness and knowledge of such an entity among health professionals is important to prevent unwarranted anxiety from misdiagnosis, and inadvertent bowel injury during cord clamping at delivery.