We report a case of esophageal leiomyosarcoma that possibly arose from the muscularis mucosae, thereby showing a particularly unusual appearance. A large polypoid intraluminal lesion in the distal esophagus was found on an endoscopic examination of a 68-year-old man with a 3-month history of dysphagia. Although the histological examination of biopsy specimens clearly revealed leiomyosarcoma, the absence of an exophytic component on computed tomography (CT) scan caused us to suspect that the tumor was carcinosarcoma. The tumor was resected by a subtotal esophagectomy. Microscopic evaluation revealed no involvement in the layer of the muscularis propria and no component of carcinoma. Clinical features of the pedunculated esophageal leiomyosarcoma shown in this case and three additional cases previously reported in the literature are reviewed.