Thoracic ectopia cordis is a rare congenital defect with very few reported survivors after surgical correction. We report a case of complete thoracic ectopia cordis with double outlet right ventricle. The diagnosis was established antenatally and a repair was undertaken soon after birth. The child remained stable and was extubated on the fifth post-operative day. Forty-eight hours later the child succumbed to an unexplained respiratory arrest. Also presented is a review of the different surgical strategies for this unusual condition.