Congenital malformations of the abdominal wall consist of a combination of a parietal opening and visceral abnormalities. The most frequent are omphalocele and laparoschisis. Embryological analysis of the formation of the abdominal wall distinguishes omphalocele, due to absence of differentiation of the lateral folds of the embryo, from laparoschisis, due to absence of differentiation of the mesenchyma at one point of the somatopleural lining. Clinically, omphalocele consists of an opening of the abdominal wall, lined by a double layer (peritoneum and external layer of the amniotic membrane), which may rupture before birth. Laparoschisis is a true antenatal hernia lying to one side of the umbilical cord. The other abnormalities of the abdominal wall are less common: muscle and purely cutaneous aplasia. The treatment of omphaloceles requires multidisplinary management. Several surgical techniques are used, mainly primary closure and rectomyoplasty. Primary closure of the abdominal wall is only possible in one-half or one-third of cases.