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BACKGROUND Leiomyoma is a rare, benign, esophageal tumor that does not often measure >10 cm. Here, we report a case of giant esophageal leiomyoma in a 24-year-old man. CASE REPORT A 24-year-old man who smoked and had primary hypertension and glucose-6-phosphate dehydrogenase deficiency presented with a history of shortness of breath and productive cough with yellowish sputum, a long history of dysphagia to solid food, and a weight loss of 7 kg over 2 months. A chest X-ray revealed a mediastinum with a width >8 cm. Computed tomography of the patient's chest revealed a multilobulated mass that originated from the upper and middle thoracic esophagus, caused severe narrowing of his esophageal lumen, and was compressing his trachea and right main bronchus. Resection of the tumor was performed and, because of the large defect after the surgery and the mucosal necrosis, the patient underwent an Ivor-Lewis esophagectomy. His postoperative course was uneventful. He had no symptoms when he was seen in the outpatient clinic for follow-up and fully recovered. CONCLUSIONS Giant esophageal leiomyoma (GEL) is a rare oncological entity that presents several diagnostic and therapeutic challenges because of the scarcity of information in the medical literature on surgical management. The descriptions of techniques for surgical resection of GEL do not include ways to effectively perform subsequent reconstruction. The aim of the present paper was to contribute to this scant information by reporting our experience with performing an Ivor-Lewis esophagectomy to manage a case of GEL.