BACKGROUND: Ectopia Cordis is defined as complete or partial displacement of the heart outside the thoracic cavity. It is a rare congenital defect with failure of fusion of the sternum with extra thoracic location of the heart. The estimated prevalence of this case  is 5.5 to 7.9 per million live births.CASE PRESENTATION:  We had a case of a 16-hour-old male neonate weighing 2.9kg with externally visible, beating heart over the chest wall. Initial treatment included covering the heart with sterile-saline soaked dressing, starting systemic antibiotics and supportive care. A staged surgical approach to this defect with the initial aim of replacement of the heart to the thoracic cavity was opted. The neonate died twenty minutes after the surgical intervention due to cardiogenic shock despite adequate resuscitative measures. CONCLUSION: This case report underscores the missed opportunity of antenatal ultra-sonographic diagnosis and the challenge of Ectopia Cordis treatment in Ethiopia. KEYWORDS: Ectopia Cordis, case report, antenatal ultrasound, Ethiopia, treatment challenge