Atypical hemolytic uremic syndrome associated with neuraminidase-producing Streptococcus pneumoniae usually associated with invasive infection such as fulminant pneumonia, sepsis, and meningitis and may occur earlier in lift and has a higher mortality rate than typical hemolytic uremic syndrome. We have experienced a 22-month-old female patient with hemolytic uremic syndrome associated with S. pneumoniae pneumonia and empyema. The patient was treated with ceftriaxone and washed red blood cell transfusion. As the disese course could be aggravated by the use of blood products containing anti-Tomsen-Friedenreich antigen, early recognition and sensible use of blood products such as washed RBC might lead to the improved outcome.