ABSTRACTThe involvement of Helicobacter pylori in the pathogenesis of extragastric diseases continues to be an interesting topic in the field of Helicobacter‐related pathology.Although conflicting findings have been reported for most of the disorders, a role of H. pylori seems to be important especially for the development of cardiovascular and hematologic disorders.Previously isolated human and animal Helicobacter sp. flexispira and “Helicobacter heilmannii” strains have been validated using polyphasic taxonomy. A novel enterohepatic Helicobacter has been isolated from mastomys and mice, adding to the list of helicobacters that colonize the liver. Genetic targets that may aid the classification of novel Helicobacter species have emerged. Animal models of Helicobacter‐induced gastric and hepatobiliary diseases have offered insights to the mechanisms associated with premalignant transformation.