Cogenital abdominal wall defects such as omphaloceles can be recognized by fetal ultrasonography. To determine whether associated anatomic features may be useful in determining fetal prognosis, a retrospective study was performed over a 5-year period. There were 28 cases of omphalocele; 16 were larger than 5 cm and classified as giant, and the remainder were considered small. Eleven of the 12 infants with small omphaloceles survived with minimal neonatal complications. Ten of the 16 infants with giant omphaloceles died because of associated congenital anomalies. These were congenital heart disease, central nervous system malformations, and diaphragmatic hernias. This review suggests that the prognosis is good when a prenatal diagnosis of giant omphalocele is made and careful fetal ultrasonography, including echocardiography, does not identify heart, central nervous system, or diaphragmatic malformations, even when there is liver herniation into the omphalocele.