Lymphangioma of the esophagus is a very rare submucosal tumor, but it can usually be removed without complication by endoscopic resection because of its small size and benign nature. My colleagues and I report a case of an uncommonly large lymphangioma (5.1 x 2.3 x 1.7 cm) in the distal esophagus treated by distal esophagectomy and esophagogastrostomy for complete resection without narrowing the esophagus. The patient's immediate postoperative course was uneventful, and he remains well 6 months after the operation.