Conjugated hyperbilirubinemia is a rare complication of hemolytic uremic syndrome (HUS). We report a case of a 2-year-old female with Streptococcus pneumonia-associated HUS (SP+ HUS) who developed severe cholestasis. It is important for pediatric gastroenterologists to be aware of manifestations of HUS, and that although rare, cholestasis can be one of the early findings in patients with SP+ HUS.