Introduction: Portal hypertensive gastropathy (PHG) is a common endoscopic finding in patients with portal hypertension (PHT). The pathogenesis of PHG is complex and a variety of etiological factors have been investigated but many controversies exist. Helicobacter pylori (H. pylori) infection role in the development of PHG is conflicting. The aim of this work: To investigate the prevalence of H. Pylori infection in infants and children with PHG and to assess the relationship between H. pylori and degree of PHG and risk of upper GI bleeding. Patients and methods: This study was conducted on 160 subjects. Eighty patients had PHT with PHG; they were recruited from the Pediatric Gastrointestinal Endoscopy Unit, Cairo University Pediatric Hospital, Egypt. Another 80 children complaining of abdominal pain or hematemesis without PHT were included as controls. All patients underwent upper gastrointestinal endoscopy to assess the severity of PHG, grade of varices and biopsies were taken from patients and controls to demonstrate H. pylori in the histopathological examination and by using rapid urease test. Results: Our data showed the prevalence of H. pylori was 61.25% in patients with PHG compared to 58.75% in the control group. There was significant association between the degree of severity of PHG and prevalence of H. pylori infection (p-value of <0.01). In patients with PHG, upper GI bleeding comparable in cases with H. pylori infection and those without. Conclusion: We found no significant difference between patients with PHT and controls as regard the prevalence of H. Pylori (p-value = 0.52). H. pylori infection among PHG patients was more associated with moderate to severe PHG.