Pentology of Cantrell (PC) is a rare syndrome with unknown etiology presenting with pentad of midline, upper abdominal wall disorder, defect of lower sternum, anterior diaphragmatic defect, pericardial abnormality and congenital abnormalities of the heart as explained by Toyama. Genetic conditions associated with PC, include trisomy 18, 13 and Turner syndrome. Prevalence varies from 1/65,000 to 1/200,000 births. Mortality depends on the size of the abdominal wall defects, ectopia cordis and associated heart defects. Early antenatal diagnosis is very much important in these cases for better management. In our case report we are presenting a neonate born at 34 weeks of gestation to an elderly multipara mother with complete PC with severe form of omphelocele major, ectopia cardis and ventricular septal defect. She had low APGAR score at birth with severe respiratory distress, was managed conservatively and expired at 24 hours of life.