Objective: To observe the endoscopic morphology of gastric varices of patients with portal hypertension type one isolated gastric varices (IGV-1) and to explore the etiology, treatment and prognosis of portal hypertension IGV-1. Methods: From January 2006 to June 2018, at Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine and North Branch of Ruijin Hospital, 54 patients with portal hypertension IGV-1 were retrospectively analyzed. The varices were classified according to the endoscopic morphology and the etiology treatment, therapeutic efficacy and prognosis were also analyzed. Descriptive method was used for statistical analysis. Results: Among the 54 patients with portal hypertension IGV-1, the endoscopic morphology of varices were tuber type in 24 patients (44.4%), grape string type in nine patients (16.7%), strip type in five patients (9.3%), dendritic type in three patients (5.6%) and mixed type in 13 patients(24.1%). Etiological analysis showed that the primary disease of 34 cases (63.0%) were hepatogenic, 11 cases (20.4%) were pancreatic origin, and nine cases (16.7%) were from other diseases. As to treatment, three cases (5.6%) were treated with adhesive, two cases (3.7%) were treated with sclerotherapy, and 49 cases (90.7%) were treated with combination of adhesive and sclerotherapy. Therapeutic efficacy evaluation showed that 46 cases (85.2%) were significantly effective, eight cases were effective, 0 case was ineffective, and all the 54 cases (100.0%) were improved. The prognostic analysis showed that 35 cases (64.8%) had no bleeding in five years and eight cases (14.8%) had no bleeding in 10 years. Nine patients (16.7%) died, including six cases of pancreatic cancer, two cases of liver failure and one case of gastrointestinal bleeding. Conclusions The endoscopic morphology of IGV-1 portal hypertension in mainly tuber type. The main cause is hepatogenic and the combination of adhesive and sclerotherapy is beneficial to the regression of gastric varices.