Umbilical cord hernia is a rare and benign congenital defect of the abdominal wall, usually misdiagnosed as omphalocele. Its association with Meckel's diverticulum is very rare, with only few cases described in literature. We present a case of a male child that was prenatally diagnosed with a minor omphalocele at 16 weeks of pregnancy. Other malformations were excluded and genetic testing was normal. The pregnancy was otherwise uneventful and the baby was born at 39 weeks without complications. Postnatally, a bulge with air and fluid content was palpable at the base of the umbilical cord stump. The baby started refusing oral feeding at 12 hours of life and the abdominal radiography showed intestinal herniation, confirming an umbilical cord hernia. Surgical exploration showed a herniated Meckel's diverticulum, and a wedge diverticulectomy and umbilicoplasty were completed without complications. In this case, the high index of suspicion warranted an early involvement of the pediatric surgery team, which contributed to the correct diagnosis before manifestation of intestinal obstruction symptoms.