In cloacal exstrophy (OEIS Complex), om­phalocele is often the initial observation at its one of the most important major findings. Other major findings include failure of visualization of the urinary bladder between the two umbilical arteries, large midline infra-umbilical anterior abdominal wall defect, low insertion of the umbilical cord, and lumbosacral meningomyelocele which usually skin-covered.

The associated minor anomalies may include lower extremity defects, renal anomalies, ambiguous genitalia, ascites, widened pubic arches, narrow thorax, hydrocephalus, cardiac defects, and single umbilical artery.

During early prenatal ultrasound Increased nuchal translucency has been reported in some cases.

Differential diagnosis: Gastroschisis, Pentalogy of Cantrell, Limb-body wall complex (LBWC), and Abdomino-schisis due to Amniotic Band syndrome.

Gastroschisis differentiated from Omphalocele by the free-floating extra-abdominal loops of bowel protrude through   paramedian defect in gastroschisis while in omphalocele the bowel loops and occasion liver herniate into a membrane-covered defect. Normal site of umbilical cord insertion in gastroschisis while in omphalocele the umbili­cal cord inserts on the covering membrane.

Pentalogy of Cantrell is the most likely diagnosis when omphalocele of the upper abdominal wall or anterior thoracic defects associated with ectopia cordis and absence of spinal defects.

LBWC is differentiated by the presence of filled urinary bladder along with severe sclerosis and fissure of the thoracic wall.

In Abdomino-schisis due to Amniotic Bands, US findings are highly variable, depending on which body part is involved and the gestational age at which the insult occurs.