Pentalogy of Cantrell is a rare syndrome which was first described in 1958 by James R Cantrell. As this syndrome is rare, it becomes more important for the radiologist to know about wide range of anomalies that can be easily detected by ultrasonography leading to early diagnosis and appropriate management. We present here a case of 23 year old primigravida whose ultrasound abdomen was done for fetal well being which showed anterior thoracoabdominal wall defect in fetus with herniation of the heart, liver and gut loops into the amniotic cavity.