PURPOSE: Helicobacter pylori (H. pylori) is recognized as the cause of primary or unexplained gastrointestinal diseases in children as well as in adults. However, it is unclear whether H. pylori causes specific clinical symptoms or diseases. Therefore, we studied the clinical manifestations associated with H. pylori infection in consecutive symptomatic children undergoing diagnostic endoscopy. METHODS: This study included 42 patients with H. pylori infection, confirmed by ELISA test, CLO test, or Warthin Starry stain of a gastric mucosa biopsy specimen. The presenting symptoms, associated disease, endoscopic findings, and hematologic features were studied in the patients retrospectively. RESULTS: The positivity of H. pylori was 15%, and increased with age. The average age of infected children was 11 years. The presenting symptoms of H. pylori infection were chronic abdominal pain (57.1%), acute abdominal pain (14.3%), pallor (11.9%), hematemesis (9.5%), and nausea/ vomiting (7.1%). The endoscopic findings were nodular gastritis (54.8%), nodular duodenitis (35.7%), duodenal ulcer (14.3%), hemorrhagic erosive duodenitis (2.4%), and normal finding (19%). The associated diseases were chronic recurrent abdominal pain (57.1%), acute gastritis (16.7%), duodenal ulcer (14.3%), and iron deficiency anemia (9.5%). CONCLUSION: H. pylori infection was associated with recurrent chronic abdominal pain, acute gastritis, duodenal ulcer, and iron deficiency anemia in children. Therefore, in patients with several gastrointestinal symptoms and iron deficiency anemia, the diagnostic and therapeutic approach of H. pylori infection is warranted.