Congenital hernia in the umbilical cord is a rare form of ventral abdominal wall defect, which, if not diagnosed, can lead to iatrogenic intestinal injuries due to improper umbilical clamping. We report a case of a newborn referred to our center for intestinal obstruction caused by a decapitated Meckel's diverticulum located within the umbilical cord. Only a few similar cases have been documented in the literature, including four fatalities. Our aim is to present this rare case to the surgical community to raise awareness about it as a potential differential diagnosis in neonatal obstruction cases and emphasize the importance of early treatment to reduce the risk of high morbidity and even mortality.