Omphalocele and gastroschisis are congenital abdominal wall defects due to improper fusion of embryonic folds during intraembryonic period. Due to this abdominal content will protrude through the defect leading to foetal mortality and morbidity. The present study explained a case of omphalocele and a rare left sided gastroschisis in the foetuses collected from Victoria general hospital and Gitam institute of medical sciences and research, Visakhapatnam. Omphalocele is associated with chromosomal anomalies but gastroschisis is an isolated condition. The embryological explanation for omphalocele is improper fusion of embryonic folds but the reason for left sided gastroschisis is early degeneration of left umbilical vein. Maternal infections, medications and smoking habits act as a predisposing factor for these conditions. Recent medical advances and neonatal care decreasing the incidence and morbidity of these congenital abnormalities