Papillary cystadenoma is a epithelial benign tumor of the salivary gland, most commonly in the oral cavity, head and neck. There was no difference in the sex distribution of patients, and the age of onset was 30-50 years old. The clinical manifestations are: slow growth local mass, long course of disease, some can be as long as decades, most patients have no obvious conscious symptoms, a few patients can appear local pain or distension. However, papillary cystadenoma of the esophagus is very rare, and few cases have been reported. We report a case of papillary cystadenoma of the lower esophagus and its treatment by endoscopy. The patient had no obvious complaint of discomfort. Routine gastroscopy found submucosal protuberant lesions in the lower segment of the esophagus (Figure 1), and endoscopic ultrasonography suggested cystic and solid lesions originating from the muscular layer of the esophageal mucosa, thus mesenchymal tumor was preliminarily diagnosed (Figure 2). After the exclusion of relevant contraindications, preoperative examinations were completed and the tumor was completely removed by esophageal submucosal tumor excision under endoscopy (Figure 3). Postoperative pathology indicated the tumor is papillary cystadenoma, and immunohistochemistry indicated that: CK7(+), CK(+), and basal cells of P63 (+) (Figure 4, 5). Due to the rare case, combined with the process of the diagnosis and treatment of this case, we reviewed the relevant reports on the papillary cystadenoma of the digestive tract in the past 60 years, in order to help clinicians to improve their understanding and treatments of the papillary cystadenoma.