The term gastroschisis is derived from the Greek word laproschisis, meaning “bellycleft.” It was used in the 19th and early 20th centuries by teratologists to designate all abdominal wall defects. Gastroschisis occurs in approximately 1 in 2,300 live births, and mortality for gastroschisis may approach 10 percent. Several maternal risk factors suspected to be associated with gastroschisis have been investigated, and there is a consistent association with young maternal age. The association of low birth weight also could be attributed to the fact that data demonstrate that the birth weights of babies with gastroschisis are significantly lower than those of the general population and are similar in different populations. Recently, Stevenson et al. proposed that gastroschisis is caused by the failure of the sac and yolk duct, as well as of the vitelline vessels, to initially incorporate to the allantois and later to the body stem. The specific role of the genetic component in the etiology of gastroschisis is unclear. Although there are reports of familial cases, gastroschisis occurs mostly as a sporadic event. It was observed that 4.7% of cases have at least one affected relative and the risk of recurrence is 3.5% among siblings. Definitive treatment is surgical. The timing and technique for surgical closure depends on the degree of intestinal inflammation, size of the defect and the newborn’s general condition. In general, the prognosis is good with a survival ≥90%, but in developing countries the risk of death may be as high as 60%.