Escherichia coli O157 is an uncommon but serious cause of gastroenteritis. This bacterium is noteworthy because a few, but significant, number of infected people develop the haemolytic uraemic syndrome, which is the most frequent cause of acute renal failure in children in the Americas and Europe. Many infections of E coli O157 could be prevented by the more effective application of evidence-based methods, which is especially important because once an infection has been established, no therapeutic interventions are available to lessen the risk of the development of the haemolytic uraemic syndrome. This Review takes into account the evolution and geographical distibution of E coli O157 (and its close pathogenic relatives); the many and varied routes of transmission from its major natural hosts, ruminant farm animals; and other aspects of its epidemiology, its virulence factors, the diagnosis and management of infection and their complications, the repercussions of infection including costs, and prevention.