Esophageal atresia with or without tracheoesophageal fistula will present with distinct clinical manifestations which serve as clue to its presence. Clinicians should be aware of them so that appropriate measures can be applied and timely referral can be made. Prevention of aspiration by constant suctioning of the proximal pouch can be life saving so is the maintenance of body temperature and provision for nutritional support Careful assessment of the clinical status and proper documentation of the anatomic defect are mandatory since the choice of surgical procedure and its timing will depend on them. (Summary)