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Background. The omphalocele represents a frequent congenital anterior abdominal wall defect with an overall morbidity and mortality that is decided by the expanse of the defect and the severity of associated anomalies. Routine prenatal screening and ultrasound diagnosis of the anterior midline defect and any associated anomalies are considered nowadays a standard of care. Moreover, its diagnosis during pregnancy requires answers regarding the long-term prognosis, motor and cognitive development, and cosmesis. Case presentation. We present the case of a 33-year-old pregnant patient, with 40 weeks of gestation, having the following obstetrical history: gestation 7, vaginal births 6, abortions 0, with the lack of prenatal care due to socioeconomic factors such as low family income and education. The patient presented at the “Elena Doamna” Clinical Hospital of Obstetrics and Gynecology, Iaşi, accusing lower abdominal pain. The obstetrical ultrasound examination revealed a 40-week pregnancy with a single viable fetus with 34 weeks and 5 days estimated age, and 1693 g estimated weight. Furthermore, it was found a defect located at the umbilical cord insertion with the sac that measured 32.8 mm, containing loops of intestines, liver and ascites. Also, it was noticed a laterodeviation of the cord with ventricular septal defect measuring 2.4 mm, and single umbilical artery. Few days later, the patient gave birth by caesarean section to a female newborn, with 2500 g weight and an Apgar score of 2-5-6. The postpartum evolution was not favorable, the newborn being transported to the pediatrics surgery department for evaluation and surgical management. The newborn died in the second day post-surgery. Discussion and conclusions. Normally, the anterior midline abdominal defect occurs at the umbilical cord, with a size between 2 to 10 cm, containing herniating midgut and various organs, such as the liver, spleen and gonads, covered by three layers (the peritoneum, the Wharton’s jelly layer, and the amniotic layer). The outcome in the presented case, which associated fetal growth restriction, was poor. [ABSTRACT FROM AUTHOR]