Omphalocele is a defect of the abdominal wall due to which evisceration of the abdominal contents enveloped by amnion and peritoneum occurs. Its prevalence is 1.4-2.7/10,000 RNV. It has a high association with chromosomopathies, genetic syndrome and other malformations that will imply a high rate of spontaneous abortions and termination of pregnancy. It is classified as a small or giant omphalocele, greater than 5cm or with liver content. Caesarean section is recommended for the giant omphalocele. The isolated omphalocele has a postnatal mortality of 10-20%. Retrospective analysis of omphalocele cases from the HUMIC Prenatal Diagnostic and Fetal Therapy Unit in the period between January 2007 and December 2019. There were 48 cases of omphalocele. The detection rate on the first trimester ultrasound was 88.6%. The mean maternal age at diagnosis was 33.7 years. There were 4 cases of omphalocele in multiple pregnancies. The abortion rate was 6.25%. 84% of the patients had an invasive technique, with 41% of fetuses presenting chromosome pathologies. Based on size, 54.2% were small omphaloceles and 45.8% were giant ones. A higher rate of chromosomopathies was observed in the group of small omphaloceles (57.5% vs. 9.1%; p <0.001). Both groups expressed a high rate of associated malformations, 80.7% vs. 68.2% respectively, with cardiac malformations being the most frequent. 11 patients continued with gestation (4 small vs. 7 giant). 72.7% were eutocic deliveries vs. 27.3% Caesarean section. There were 2 successes: one corresponds to a 25-week premature fetus and the other to 3 months of life due to complications after multiple surgeries. All the small omphaloceles resolved with a single surgery, while 85% of the large omphaloceles required 2 or more surgeries. Omphalocele is a diagnosable pathology in the first trimester with a high association with chromosomopathies and other malformations that makes multidisciplinary management of these fetuses necessary.