A 77-year-old man visited our hospital because of dysphagia. Endoscopy revealed a flat and slightly reddish lesion in the middle esophagus (A). Narrow-band imaging (NBI) showed a brownish area (B). Magnified NBI showed a typical type B1 pattern of intraepithelial papillary capillary loops (C). Pathologic examination of biopsy specimens revealed a high-grade intraepithelial neoplasia. Computer tomography showed no abnormalities. An en bloc resection was performed by endoscopic submucosal dissection. Microscopically, the lesion size was 38 × 17 mm. Histopathologic examination revealed a component of squamous cell carcinoma in the mucosal surface, whereas epithelioid tumor cells were present as cribriform, tubular, and solid patterns in the lamina propria, which had no relation to the superficial squamous epithelium (D). Immunohistochemically, the tumor cells expressed P40, P63, SMN, S100, CK5/6, and CD117. The final diagnosis was early type 0-IIb esophageal adenoid cystic carcinoma (EACC) accompanied by squamous cell carcinoma (pT1b). The vertical and horizontal edges of the resected lesion were negative. There was no lymphovascular or neural invasion of cancer cells. Endoscopy 3 months after resection showed that the lesion was completely healed, with no recurrence.