Abstract:Gastroschisis is a ventral body wall defect causing herniation of abdominal contents through para-median abdominal wall defect. The incidence is 1/20,000 live births. Various hypotheses are proposed but most of the authors support the hypothesis of failure of mesoderm to form body wall. Gastroschisis typically occurs to right of umbilicus, with very few cases of left sided gastroschisis reported in literature. We report a left lateral gastroschisis with concurrent complete liver agenesis, rudimentary left lung and left pulmonary artery.