Ectopia cordis is a rare heart lesion that results from an abnormal development of the primitive heart outside the embryonic disc in the early stage of development. It represents a form of pericardial defect but is further characterized by a partial or complete displacement of the heart outside the thorax. Congenital heart diseases like tetralogy of Fallot (most common), ventricular hypoplasia, transposition of great vessels (TGA), tricuspid atresia, Ebstein's anomaly, common atrium, may be present. Multiple extra-cardiac defects have also been occurs this like with meningocele, encephalocele, cleft lip, palate deformities and ventral wall defects. The prognosis is very poor in these cases.