Enterohemorrhagic Escherichia coli (EHEC) have emerged over the last decade as important enteric pathogens because of their potential to induce both hemorrhagic colitis and fatal hemolytic uremic syndrome (HUS). HUS following EHEC colitis has become the leading cause of pediatric renal failure requiring kidney transplant in North America. The ability for EHEC to induce disease is dependent upon their ability to adhere to the intestinal mucosa in an intimate fashion, and to produce potent cytotoxins. These virulence factors (toxin production and enteroadherence) have been implicated in the pathogenesis of EHEC-induced disease. In this review we will discuss the symptomatology, epidemiology, laboratory diagnosis, pathogenesis, complications, treatment, and prevention of EHEC disease. We will review HUS with emphasis on treatment and prevention. Finally we will review animal models for EHEC infection in order to discuss their role in developing new strategies for the treatment and prevention of EHEC-associated diseases.