A rare case of adenoid cystic carcinoma of the esophagus is reported. A patient was a 48-year-old woman was admitted to the hospital because of dysphagea. Esophagography revealed a lesion at Ei, and an endoscopy demonstrated an uneven elevated lesion on the anterior wall of the esophagus 30-38cm from the incisors. An adenoid cystic carcinoma was diagnosed by a biopsy and a total thoracic esophagectomy was performed. The surgical material showed a 6.5×4.0cm 2type lesion with an ulcer in the center. The histopathological diagnosis of esophageal adenoid cystic carcinoma (mp, n2, stage III) was made. Immunohitological staining for actin was positive in the tumor. Electron micrograph showed microvilli. Esophageal adenoid cystic carcinoma is generally thought to be associated with a poor prognosis, but the patient has not developed recurrence and has been followed as an outpatient as of three years after surgery.