Ectopia cordis is a rare congenital defect in fusion of the anterior chest wall resulting to extrathoracic location of the heart. It may occur as an isolated malformation or can be associated with a larger category of ventral body wall defects that affect the thorax, abdomen or both. Imaging clues of ectopia cordis on frontal chest radiograph include abnormal cardiac position (usually midline) and configuration, air lucency surrounding the ectopic heart, sternal defect, wide separation of the sternal ends of the clavicles and widening of the superior mediastinum.  Lateral view may confirm the extrathoracic location of the heart.