Background: gastroschisis is one of the variant of anterior abdominal wall defect. Gastroschisis commonly present on the right side of the umbilical cord. Left sided gastroschisis is extremely rare entity. Both right and left gastroschisis located in periumbilical region. Case report –a 1-day old male baby weighing 2300 gram admitted at our institution. His bowel was exposed and coming out through anterior abdominal wall defect. The abdominal wall defect was located left lateral to umbilical cord. Conclusion –this index case of left gastroschisis representing here due to rarity.