Rare congenital problems generally have no standardized diagnostic or operative technique. Because of some variation within the spectrum, diagnosis and management has to be individualized and tailored according to the local findings to relieve the symptoms. We report a diagnostic dilemma and surgical management of a child with proximal tubular duplication of esophagus with complete remission of symptoms after surgery. To diagnose rare malformations a high index of suspicion is required when differential diagnoses are being considered.
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