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.
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