Congenital anterior abdominal wall defects encompass numerous anomalies. What all of them have in common is the protrusion of abdominal organs outside of the abdominal cavity. Most commonly, it is the case of protrusion of intestines. Anomalies occur during the early embryonic development, amidst the absence of physiological umbilical herniation withdrawal. The most common anomalies are omphalocele and gastroschisis, with the incidence of around 3 cases on 10 000 live births. The main difference between them is that in the case of omphalocele, the abdominal content is covered with a sac. Conversely, the intestines are not covered in the case of gastroschisis. Furthermore, omphalocele is often linked to chromosomal anomalies, which is not the case with gastroschisis. Besides of the aforementioned anomalies, the ones that are not as common are pentalogy of Cantrell, bladder exstrophy and body stalk anomaly. Pentalogy of Cantrell is more complex because, in addition to abdominal wall defects, anomalies of sternum, heart, pericardium and diaphragm can also be found. Bladder exstrophy represents the most common version of the bladder exstrophy - epispadia complex (BEEC), in which the anterior bladder wall is missing while the posterior wall is exposed. Body stalk anomaly is the most severe form in which malformations of the thoracic and abdominal walls are present, with a plethora of associated anomalies and it almost always ends in intrauterine demise. Prune belly syndrome encompasses a triad of symptoms including deficiency of abdominal muscles, abnormalities of urinary tract and bilateral cryptorchidism at boys. It is most commonly found in males. The diagnoses of the aforementioned anomalies are set at the end of first or at the beginning of the second pregnancy trimester. Surgical procedure forms a basis for treatment.