Objective To investigate the clinicopathological features of gastric adenocarcinoma of fundic gland type (GA-FG). Methods A total of 12 patients, including 7 cases treated with endoscopic submucosal dissection (ESD), were diagnosed as having GA-FG in Nanjing Drum Tower Hospital from January 2018 to August 2019. Morphological changes were analyzed by reviewing endoscopic and pathological results. Patients were followed up after definitive diagnosis. Results The clinical symptoms of patients with GA-FG were nonspecific. No Helicobacter pylori infection was identified. The lesions were found in the non-atrophic gastric mucosa of the upper 1/3 portion in 10 cases and middle 1/3 portion in 2 cases. Endoscopically, the most common features were whitish color (9 cases), and all lesions diameter≤1 cm. Their macroscopic types were classified as 0-Ⅰ (2 cases), 0-Ⅱa (9 cases) and 0-Ⅱc (1 case) respectively. All lesions had sharp boundary, with branching dilated blood vessels on the surface. Five in 7 cases who were treated with ESD showed submucosal invasion. Immunohistochemically, 9 cases were classified as the chief cell type , 3 as the mixed type, 11 MUC6 positive, 4 MUC5AC positive, 2 MUC2 positive, and 3 CD10 positive. P53 was detected in all 12 cases, and 9 cases had low Ki-67 staining index (<10%). The mean time of follow-up was 11 months, and 11 patients survived. Conclusion GA-FG should be taken into consideration when the polyps are found in the upper part of the stomach, with whitish color, and branch dilated blood vessels on the surface. Excellent clinical outcomes can be achieved for GA-FG patients with ESD.