Hemolytic uremic syndrome (HUS) is a disease of the vascular endothelium characterized by a triad of hemolytic anemia with the presence of schizocytes, thrombocytopenia, and acute kidney injury. The majority of cases are associated with infection by Escherichia coli that produces verotoxins or shigatoxins. Transmission can occur through food, person-to-person contact, or contact with infected ruminants. After entering endothelial cells, these shigatoxins induce a cytotoxic effect responsible for apoptosis and a pro-inflammatory response. Since 1996, the French Institute for Public Health Surveillance has been compiling cases diagnosed in patients under 15 years of age treated in pediatric nephrology units within a voluntary surveillance network in mainland France and initiates exploratory investigations in response to clusters of cases. The annual incidence in France ranges from 0.6 to 1 per 100,000 children under 15 years of age, with a peak at age one. Fifty-one percent of HUS cases occur between June and September. Management is symptomatic, with extracorporeal renal replacement therapy used in 46% of cases in 2009; peritoneal dialysis should be preferred. Neurological complications represent the most common extrarenal involvement and the leading cause of death; early plasmapheresis may improve the prognosis. Mortality ranges from 1% to 5%. One-third of patients experience renal sequelae: proteinuria, hypertension, and reduced glomerular filtration rate. The risk of sequelae increases with the duration of anuria. All patients who have had HUS must undergo lifelong monitoring.