Pentalogy of Cantrell is a rare congenital anomaly consisting of the following features: (1) midline supraumbilical abdominal wall defects (2) deficiency of the anterior diaphragm (3) defects in the diaphragmatic pericardium (4) defects of the lower sternum (5) congenital cardiac malformation We report a case of Cantrell's Pentalogy with rare cardiac malformation single ventricular chamber with two atria ventricular valves and pulmonary stenosis. The newbom had multiple congenital anomalies: omphalocele, thoracoabdominal ectopia cordis, diaphragmatic defect, short sternum/sternal defect and dysmorphic features--microcephaly, hypertelorism, down slanted palpebral fissures, micrognathia, low set ears.