Omphalocele (exomphalos) is one of the most common abdominal wall defects. The size of the defect and the severity of the associated anomalies determine the prognosis, the morbidity and mortality of this pathology. Prenatal screening and diagnosis of the abdominal wall defect and concurrent anomalies is important as it allows for effective prenatal counseling and optimal perinatal management. The purpose of this article is to discuss the approach to prenatal diagnosis and management of omphalocele through two cases reported.