The precise etiology of hemolytic uremic syndrome (HUS) is unknown. However, it has been associated with bacterial (Shigella, Salmonella, E. coli, S. pneumoniae), Bartonella, and viral (coxsackie, ECHO, influenza, varicella, Epstein-Barr) infections and with endotoxemia. Recently, we experienced a case of HUS in a 16-year-old boy who was in the acute phase of an Epstein-Barr virus (EBV) infection. He had typical manifestations of HUS and EBV infection. He also transiently presented disseminated intravascular coagulation. His renal dysfunction recovered by supportive care, including hemodialysis, plasmapheresis, antihypertensive medication and aspirin. We present this case with a review of the literature as the second report of HUS associated with EBV infection.