Omphalocele is a midline anterior wall defect that allows herniation of abdominal contents into a normally situated umbilical cord base insertion. The etiology is thought to be secondary to migration failure of the lateral mesodermal body folds or persistence of the primitive body stalk. With an incidence of 1:3000 –5000 live births there is an increased risk of associated anomalies such as: chromosomal defects (Trisomy 13, 18, 21), cardiac anomalies, genitourinary and central nervous system. Omphalocele is also associated with Beckwith-Wiedemann syndrome which is an autosomal dominant disorder characterized by gigantism, macroglossia, and pancreatic hyperplasia. In omphalocele the hernia is always midline and contained within a sac. The contents may be bowel, mesentery or even liver. This is in contradistinction to gastroschisis which is an isolated evisceration of the intestine through a right-sided paraumbilical wall defect and rarely associated with other anomalies. Since omphaloceles are associated with other anomalies, not only should a detailed and careful search be undertaken, but also chromosomal evaluation should be performed in utero (amniocentesis). Surgical correction after a planned delivery at a tertiary medical center is the mainstay of treatment. Most cases of omphalocele can be delivered vaginally unless this is otherwise contraindicated.