An umbilical cord hernia (UCH) is a form of abdominal wall defect, affecting 6 out of every 10,000 newborns. The persistence of urachus is an embryonic remnant that connects the bladder to the abdominal wall at the level of the umbilicus, being yet more uncommon. We reviewed the literature, searching in PubMed, under the terms “Hernia of umbilical cord”, “Congenital hernia of cord” and “Persistent Urachus”. Only a few similar cases of both pathologies associated described were found. Our main objective is to highlight the distinct clinical features, embryogenesis, prognosis and associated anomalies of two infrequent embryopathies. And to describe a infrequent case of both abnormalities presenting simultaneously.

UCH are often misdiagnosed with other abdominal wall deffects, such as omphalocele, umbilical hernias, gastroschisis and umbilical cord cysts. The normal cord insertion, adequate muscle development of the abdominal wall and a wall defect less than 5cm is what differentiates it from an omphalocele. UCH has a low morbidity overall, as it is not associated with other anomalies. The most frequently observed urachal malformations are the persistence of a urachus and urachal cyst. The prenatal diagnosis of patent urachus is made by ultrasound or magnetic resonance, being easily mistaken with abdominal wall defects, confirming the diagnosis with an ultrasound at birth. The persistence of urachus may resolve spontaneously, if not, surgical resolution is recommended. Similar to a UCH, a patent urachus shows little association with other malformations.

It is important to know the clinical presentation and the diagnostic perinatal methods employed for appropriate management and favorable results for both pathologies. This relies on knowing when to suspect possible associated anomalies and when complementary studies might be needed. It is also important to be aware that there is the possibility of a UCH and a patent urachus existing simultaneously.