Leiomyosarcomas are uncommon tumors, and even rarer are esophageal leiomyosarcomas. For patients with respectable esophageal leiomyosarcomas, standard treatment is usually esophagectomy. Herein, however, we report a case of pedunculated esophageal leiomyosarcoma that was successfully removed by local excision without esophagectomy, which is interesting in that only four such cases of local excision of esophageal leiomyosarcoma has been reported. Our patient underwent postoperative irradiation and, as of his two-year follow-up, was still alive and without symptom. After describing the case, we review the literature on the clinical behavior and management of leiomyosarcoma. In cases of well-defined esophageal leiomyosarcoma, local excision might be as effective as radical resection and have fewer postoperative complications and less morbidity.