Pentalogy of Cantrell is a rare multiple congenital malformation syndrome characterized by a combination of features: a midline supraumbilical abdominal wall defect, a defect of the lower sternum, a defect of the diaphragmatic pericardium, deficiency of the anterior diaphragm, and congenital cardiac anomalies. This congenital defects could be diagnosed as early as in the first trimester of pregnancy. The complexity of this anomaly, especially coexistent heart defects, determines the way and order of surgical treatment and commonly is a prognostic factor. A case of male newborn with prenatal diagnosed omphalocele and diaphragmatic hernia is reported in the present work. The child was operated within two hours after birth. Intraoperativelly a significant defect of diaphragmatic pericardium and heart surrounded by a small bowel were discovered. The defect of diaphragm was sutured and a drainage of left pericardial cavity was put in place. Afterwards, the abdominal wall defect was sutured after transferring organs into a peritoneal cavity. Six days after the procedure cardiac sonography was performed and it indicated the presence of hemodynamically insignificant congenital intracardiac defect – atrial septal defect. Up-to-date psychomotor development of the boy, currently five months old, is correct. Prenatal diagnosis of the complexity of congenital defects and possibility of fetus condition monitoring allows to plan precisely the time and way of the delivery, as well as to decide the treatment method of the newborn. Due to the absence of hemodynamic disorders and in spite of the present cardiac defect, an early surgical treatment of our patient was possible.