A review of the world literature yielded 95 cases of leiomyosarcoma of the esophagus. Another case has been added. The symptoms of esophageal leiomyosarcoma and squamous cell carcinoma are approximately the same. This rare tumor tends to be polypoid, rarely invasive and metastatic. Surgical resection with esophagogastric anastomosis is the best treatment, with a 5-year survival rate of 27.4%.