The patient was a 53-year-old man who presented because health screening examination had revealed an asymptomatic abnormality of the esophagus. Esophagography revealed a lesion at Im-Ei, and endoscopy demonstrated an uneven elevated lesion on the anterior wall of the esophagus 30-35 cm from the incisors. Biopsy gave a diagnosis of squmous cell carcinoma and total thoracic esophagectomy was performed. The surgical specimen showed a 4.3×1.5 cm 0-I sep lesion with an uneven surface featuring a reddish concavity. The histopathological diagnosis was esophageal adenoid cystic carcinoma (sm, stage 0). The postoperative course was favorable, and adjuvant chemotherapy was performed. After about 3 years of follow-up postoperatively, the patient has not developed recurrence and continues to be reviewed as an outpatient. Esophageal adenoid cystic carcinoma is generally thought to be associated with a poor prognsis, but death due to recurrence is more frequent in patients wih stage II or more advanced disease. Tumors measuring 2 cm or more in diameter are likely to be accompanied by lymph node metastasis, but 93% of superficial carcinomas are n(-). With stage 0 disease, a good prognosis can be expected.