In recent times with development of advanced ultrasound equipment and techniques early detection of various fetal anomalies are made easier. Dedicated sonographic technique and interpretation is necessary for early diagnosis of various anomalies. Omphalocele is a type of anterior abdominal wall defect, which is an infrequently reported rare congenital deformity, if detected earlier can help in emergency intervention which can be carried out for a better integrated management for fetus, Mother and family. Special importance needs to be made for Omphalocele as they are associated with multiple other jeopardizing congenital defects leading to poor prognosis. In this case report, we present antenatal imaging findings & gross fetal specimen correlation of fetus of a 21 week primigravida with Omphalocele, Cleft lip and cleft palate, Ebstein’s anomaly with hypoplastic left heart, Enlarged posterior fossa of brain with hypoplastic cerebellum and vermis, Large cisterna magna, Hypoplastic orbits and enlarged hyperechoeic kidneys. Of all the investigations, ultrasonographic assessment of fetal structure and well being is most reliable along with other investigations like genetic screening. The lack in the dedicated antenatal examination and poor compliance for routine antenatal checkups leads to a large proportion of undiagnosed cases. Marked reduction in perinatal morbidity and mortality and avoidance of stigma faced by the parents can be achieved by early detection and proper management.