A case of lymphangioma of the esophagus with symptoms of achalasia was presented. Compressible and folded intramural tumors were found from the middle to the lower part of the esophagus and the Mecholyl test was positive. After incisional biopsy at lower esophagus through full thickness of the wall, the opening was repaired with Thal-Hatafuku's fundic patch procedures to relieve achalasia. Postoperative course was uneventful and symptoms of achalasia completely disappeared.