Experience in 100 patients with esophageal leiomyoma treated surgically at the authors’ Department during the past 27 J years was reported. During the same period of time approximately 3000 patients with esophageal carcinoma and 5 patients with esophageal leiomyosarcoma were also operated upon at this Department. Malignant transformation of esophageal leiomyoma under observation had not been recorded in the authors’ experience. Of the 100 patients preoperative diagnosis was straightforward in 90 and difficult in 10, being misdiagnosed as carcinoma in 4, mediastinal tumors in 3, bronchial cysts in 2, and leiomyosarcoma in 1, a fact indicating that the diagnosis of esophageal leiomyoma is by no means easy. Preoperative esophagoscopy revealed normal mucosa in all but 2 cases, of which one showed deep ulceration and necrosis of tumor tissue and the other a small ulcer of the mucosa at the site of previous biopsy. In the present series the tumor was located in the upper segment of the esophagus in 15, middle segment in 71, and lower segment in 14. It was single in 93 and multiple in 7. In 99 of the patients the tumors were successfully removed by extramucosal enucleation. In only 1 case, due to misdiagnosis for carcinoma, was partial esophagectomy performed. Rupture of mucosa at enucleation of tumor occurred in 7 cases, all repaired by simple suturing. The causes of mucosal rupture were very large and irregular tumors in 2, multiple tumors in 1, preoperative irradiation in 1, previous endoscopic biopsies in 1, and operative inadvertence in 2 of the cases. The only one death in this series occurred in a patient who had enucleation with rupture of mucosa which was repaired, and who developed leakage and empyema postoperatively and terminated in fatal hemorrhage due to perforation of thoracic aorta 18 days after operation. Long term follow up study in 65 patients showed no recurrence of tumor. All patients enjoyed good quality of life. No symptoms related to reflux esophagitis were observed. Barium swallow in 50 patients showed mild narrowing of esophageal lumen in 3 and small diverticulum in 1, all at the site of previous enucleation, and normal findings in the remaining 46. Experience in this study showed that in surgery for leiomyoma of the esophagus extramucosal enucleation is practically always feasible and should be the procedure of choice. It was also stressed that avoidance of endoscopic biopsy, avoidance of mucosal rupturing, careful examination for presence of mucosal perforation, and meticulous repair of mucosal rupture, if present, to avoid postoperative leakage and fistula formation, are all important measures to minimize operative morbidity and mortality for leiomyoma of the esophagus.