Esophageal atresia occurs in 1 out of 2500 to 4500 live births; an isolated variant occurs in 8% of the cases. In this case report and literature review, we present a rare case of isolated distal esophageal atresia happening just 2 cm above the diaphragmatic right leaflet with the connecting band between the two esophageal parts. The location raised the possibility of a direct vascular accident to that segment as a cause of the atresia. Post-operative ambu bagging may facilitate leak at the anastomosis site and should be done carefully, as well as, to introduce contrast with suckling not with jet injection for the same reason. We mentioned that to raise a wariness as this mishap may cause problems.