Review of the world literature to the end of 1971 has provided data on 838 cases of esophageal leiomyomata, including our own 19 surgically removed lesions. Although esophageal leiomyoma is the most common of the benign tumors of the esophagus, it is still rare compared with carcinoma. It occurs in more men than women, by a ratio of 1.9 to 1. Over 50% of the patients with leiomyoma of the esophagus are asymptomatic. Dysphagia and vague pain are the most frequent symptoms. Pyrosis is mentioned in the literature as present in 40% of the cases, but it is considered mainly as symptom of coexistent hiatal hernia. Diagnostic problems often arise, as the smooth muscle tumors may mimic mediastinal neoplasms, cysts, or even aneurysms, or complicate coexisting hiatal hernia and esophageal diverticulum. Operative management by transthoracic enucleation is the procedure of choice, although resection of the esophagaus may be required in few cases. Postoperative morbidity is minimal and results are excellent.