Background: Ectopia cordis is a rare congenital anomaly characterized by partial or complete displacement of the heart outside the thoracic cavity. Here, we report a case of two-day-old black neonate born with an ectopia cordis associated with an omphalocele. Case Report: A full-term, barely responsive, black newborn presented at the pediatric surgery department with a heart totally outside the thoracic cavity without pericardium protection and an omphalocele. The baby was transferred immediately to the intensive care unit but died a few hours later before any surgical treatment. Conclusion: Ectopia cordis is an uncommon congenital anomaly. Its management represents a challenge even in developed country, and its prognosis is poor despite advances in neonatal cardiac surgery.