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Helicobacter pylori (H. pylori) has been identified as the major agent in human gastric cancer by the International Agency for Research on Cancer (IARC). Infection caused by H. pylori plays a leading role in many disorders including duodenal and gastric ulcer, chronic gastritis, lymphoid tissue lymphoma, and gastric adenocarcinoma. In addition, growing evidence suggests that H. pylori interferes with many biological processes, causing or affecting the incidence of several extra-gastrointestinal disorders. The bacteria are known to cause iron deficiency anemia (IDA), vitamin B12 deficiency, and immune thrombocytopenic purpura (ITP). Latest studies suggest that H. pylori may contribute in many disorders such as insulin resistance, acute coronary syndrome, neurological diseases among others, which previously was attributed to other factors and conditions. There are several mechanisms proposed for H. pylori inducing low-grade chronic inflammation and the incidence of molecular imitation mechanisms. This present study discusses the most critical diseases related with the role of H. pylori and related infection (especially extra-gastrointestinal diseases) in these diseases.