Hemolytic uremic syndrome associated with infection by neuraminidase-producing Streptococcus pneumoniae usually presents with fulminant pneumonia and has a high mortality rate. Post-pneumococcal hemolytic uremic syndrome may occur earlier in life than classical hemolytic uremic syndrome. We describe an 18-month-old male with hemolytic uremic syndrome, S. pneumoniae pneumonia, and T-antigen activation characteristic of neuraminidase activity. We have summarized the features of this case and 11 previously reported children. As the use of blood products containing IgM may aggravate this disorder, early recognition of hemolytic uremic syndrome associated with S. pneumoniae neuraminidase production may lead to improved patient outcome through the judicious use of blood products.