Leiomyosarcoma of the esophagus is a rare malignant tumor with slow growth and late metastasis. The aim of this study was to reassess the clinical characteristics and treatment modality in one of the largest series of esophageal leiomyosarcomas from a single institution. From February 1973 to December 2011, 12 cases of esophageal leiomyosarcoma were identified. The principal symptoms included progressive dysphagia in 11 cases (91.7%), retrosternal/back pain in four (33.3%), weight loss in four (33.3%), upper gastrointestinal hemorrhage in two (16.7%), and emesis in two (16.7%). The average duration of symptoms was 10.6 months. The location of the primary tumor was in the middle thoracic esophagus in five cases, and lower thoracic esophagus in seven cases. Six cases were classified as the polypoid type, five cases as the infiltrative type, and only one case as the intramural type. All 12 of the patients underwent esophagectomies, and radical resections were achieved in these patients. Based on the Kaplan–Meier Method, the 3-, 5-, and 10-year survival rates were 80.0%, 58.3%, and 31.1%, respectively, with a median survival of 63 months. Five-year survival rates for patients with polypoid or intramural tumors (n = 7) was 83.3%, and for patients with infiltrative tumor (n = 5) it was 25.0%. One of the patients had tumor resected four times and survived for 161 months. In conclusion, patients presenting with esophageal leiomyosarcomas have an excellent prognosis, and radical resection may achieve acceptable results.