Adenoid cystic carcinoma (ACC) is common in the salivary glands but rare in the esophagus. Routine esophagogastroscopy performed in a 54-year-old woman as part of a medical check-up revealed a submucosal tumor (1.5x1.0 cm) at the mid-esophagus. Endoscopic ultrasonography revealed a lesion with mixed echogenicity in the submucosal layer. The submucosal mass was removed by incisional endoscopic enucleation, and pathological analysis revealed epithelial cells with small hyperchromatic angular nuclei in tubular and cribriform patterns. The lesion was pathologically confirmed as an ACC of the esophagus.
