Background: With the advancement of ultrasound (US) tools and methods, it is now simpler to identify different fetal abnormalities early on. A structural deficiency in the front abdominal wall causes the intestines or other abdominal organs covered by a protective membrane to protrude in omphalocele, an uncommon congenital condition. Objectives: We report a case of omphalocele with liver herniation for better diagnosis and management. Patient and methods: At 36 weeks' gestation, a 41-year-old multigravida lady arrived at El Taisir Hospital in preparation for labor. The case was subjected to US. Results: US detected a male fetus with an estimated weight of 2,700 and a normal fetal heart rate of 130 bpm with a large anterior abdominal wall defect with herniated large sac covered by membrane containing liver and bowel loops. Trisomy 13, 18, and 21 are linked to 20% to 50% of omphalocele occurrences, which lead to fetal death. An increased maternal blood alpha-fetoprotein concentration or a late first trimester US or anomaly scan with a 75% sensitivity and 95% specificity can be used to diagnose omphalocele in utero. When the abdominal wall fails to close during the first month of pregnancy, large omphaloceles with liver herniation can result in. Compared to individuals without a herniated liver, omphalocele with a herniated liver showed a lower frequency of congenital abnormalities. However, it is associated with more potentially fatal anatomical and amniotic volume anomalies. Conclusion: To help with the implementation of the best treatment plan and elective pregnancy termination, prenatal diagnosis of this entity and assessment of the defect size and other related abnormalities are required. Surgery is typically done right after birth for small or minor omphaloceles, reducing the omphalocele's contents and essentially closing the defect. Large or major omphaloceles are frequently repaired in phases, beginning with the covering of the exposed organs with a non-adherent bandage. Before being surgically closed, the organs were progressively moved back into the abdomen. It is important to keep the baby's temperature stable.