Infection with H. pylori causes chronic gastritis and is the most important risk factor for gastric cancer. Besides gastric disease, infection is also thought to play a role in the development of other human diseases. H. pylori has been predominantly associated with gastrointestinal, metabolic, cardiovascular, respiratory, and neurological disorders. In the past year, novel evidence has demonstrated a significant impact on human morbidity and mortality, mostly derived from association studies. In addition, novel experimental data have revealed direct and indirect mechanisms through which H. pylori affects extragastric organs. This article provides an update on the current knowledge of extragastric diseases associated with H. pylori infection.