Seven cases of leiomyoma and two cases of leiomyosarcoma of the esophagus were presented. Review of the Japanese literature revealed a total of 260 cases of the former and only 35 cases of the latter, including our own cases. They occurred more frequently in males than females, by a ratio of 1.6:1 and 1.8:1, respectively. The patients with leiomyosarcoma distributed in higher age groups. The most frequent symptom was dysphagia in both groups of the cases, but in cases of leiomyosarcoma the incidence was much higher and the duration of the symptom was shorter. About 20% of the patients with leiomyoma were asymptomatic. Although the size of leiomyosarcoma tended to be larger, giant leiomyomas more than 10 cm in diameter were found in 18% of the cases. The lower third of the esophagus was the site of both tumors in over half of the cases, the middle third being the next frequent. The surgical removal by enucleation was the procedure of choice for leiomyoma. However, when either the tumor is densely adherent to the mucosa for considerable length or accidental extensive mucosal tear occurs, esophageal resection is recommended. Postoperative morbidity was minimal and results were generally excellent. On the contrary, esophageal resection including the tumor was indicated for leiomyosarcoma. The long-term results were poor particularly in cases of infiltrating-type lesions, though survival data could not be fully evaluated.