Leiomyoma is one of the most commonly observed esophageal submucosal tumors, often appearing as a smooth-surfaced and semicircular protruded lesion. It sometimes grows toward the esophageal lumen and may be pedunculated in rare cases. We encountered a case of a pedunculated esophageal submucosal tumor diagnosed before treatment as a leiomyoma originating in the muscularis mucosae of a 68-year-old man. As the tumor arose in the muscularis mucosae, it could be safely resected via an endoscopic procedure. Only one case of pedunculated leiomyoma has been reported to date, and we herein report the second case, which was successfully treated by a minimally invasive endoscopic technique.