Leiomyosarcomas of the esophagus are rare, malignant, smooth-muscle tumors. The presenting symptoms are indistinguishable from other esophageal neoplasms, though the history may be longer due to the slow growth of these tumors. Barium studies may show large intramural masses with ulceration or tracking, expansile intraluminal masses or areas of luminal narrowing. Endoscopic biopsies may give a high false negative rate especially in cases where the mucosa is intact. The treatment of choice is surgical excision. Synchronous and metachronous metastases do not preclude surgery, provided the metastases are also resectable. Prognosis is better than in patients with squamous esophageal cancer. The role of adjuvant radiotherapy and chemotherapy is controversial. We report a 40-year-old man who presented to us with dysphagia and was found to have a leiomyosarcoma of the esophagus. He was treated successfully with esophagectomy and is disease-free after 7 years. We review the literature on esophageal leiomyosarcomas and their management.