Gastroschisis is a congenital abdominal-wall defect that typically occurs to the right of the umbilicus. Only twenty-one cases of left-sided gastroschisis have been described in the literature. Here we report a large left-sided gastroschisis with pulmonary hypoplasia, scoliosis, ventricular septal defect and absence of gallbladder. Section of placental membranes revealed vacuolization of the amnion, without increased macrophage infiltration of the chorion. Postmortem comparative genomic hybridization micro array did not identify a specific genetic abnormality. Some of the previously reported cases were complicated by additional abnormalities and comparisons with these cases are discussed.