Leiomyoma is the most common benign tumour found in the esophagus but it is, however, a rare neoplasm; in fact of all esophageal tumours, benign tumours account for fewer than 10%, of which 4% are leiomyomas. Leiomyomas should be removed when diagnosed, even if asymptomatic, because malignancy cannot otherwise be excluded and symptoms are likely to develop if treatment is delayed or omitted. Enucleation of esophageal leiomyoma is a safe and effective procedure. We report a case of symptomatic giant anular leiomyoma of the distal esophagus, compressing the trachea and the descending aorta, resected after right thoracotomy.