Omphalocele is the commonest fetal abdominal wall defect and can represent a part of a syndrome or an isolated defect. The eviscerated abdominal organs are covered by the peritoneum and amnios. Obstetric prenatal ultrasound should be integrated by fetal echocadiography because of high incidence of associated cardiac anomalies. The authors describe the diagnosis and management of a 41 years primigravida woman with first trimester ultrasound diagnosis of omphalocele.