We present a case of a giant (13-cm length) purely polypoid esophageal leiomyoma without any intramural development. This form of leiomyoma is rare and develops due to proliferation originating from the muscularis mucosae, although the intramural type originates in the muscularis propria. This should not be confused with giant fibrovascular polyps, which are postulated to arise at the pharyngoesophageal junction when a flap of mobile, redundant submucosa prolapses distally and may cause asphyxia when protruding into the mouth. Our case was successfully removed by a right thoracotomy.
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