Pentalogy of Cantrell is a rare combination of midline developmental defects. This malformation syndrome, which was first described by Cantrell et al in 1958, is characterized by five distinctive congenital defects: supraumbilical thoracoabdominal wall (omphalocele, diastasis recti), lower sternum, diaphragmatic pericardium, anterior diaphragm, and cardiac anomaly. Ectopia cordis is another rare, fatal abnormality characterized by complete or partial displacement of the heart outside the thoracic cavity, and it can often be seen as a component of the pentalogy of Cantrell. We describe a male neonate who had complete ectopia cordis, omphalocele, and sternal, diaphragmatic and pericardial defect. The pulsating heart and major vessels could be seen outside the thoracic cavity. He had cyanosis and was intubated soon after birth, but developed pneumothorax and died on the third day of life. By presenting this very rare and dramatic case, we emphasize the importance of prenatal diagnosis and the necessity of surgical experience in developing surgical techniques.

Keywords: Cantrell pentalojisi, ektopia kordis, orta hat gelişim bozukluğu.