Background. The hemolytic-uremic syndrome (HUS) comprises hemolytic anemia, acute renal failure, and thrombocytopenia. It is the most frequent cause of acute renal failure in children. Methods. This review is based on an extensive overview of the literature dealing with the HUS in children. Results, HUS is the most common cause of acute renal failure in infants and young children and follows a diarrheal prodrome approximately 90% of the time. Nearly all postdiarrheal cases are caused by enterohemorrhagic E coli infections, in particular serotype O157:H7. Mortality is around 5%, and approximately 50% of survivors manifest some types of sequelae. Conclusion. Surveillance and contact investigation are important to control outbreaks, as well as early and aggressive treatment of symptomatic subjects to prevent mortality and severe complications, such as chronic renal disease.