Superimposed spontaneous esophageal perforation in congenital esophageal stenosis

Congenital esophageal stenosis (CES) is a very rare embryologic anomaly of tracheoesophageal development that requires lifelong management, usually with frequent esophageal dilations and dietary precautions. We present a patient with spontaneous thoracic esopahgeal perforation in a setting of CES. The patient, a 27-year-old male, recovered uneventfully following open primary esophageal closure. A brief but comprehensive discussion of CES follows the case report.