A previously healthy young female, presenting with nausea, vomiting, diarrhea, anemia, thrombocytopenia, and acute renal failure, was admitted to our hospital. Her clinical and histological features were consistent with both hemolytic uremic syndrome and IgA nephropathy, and she responded to steroid treatment, plasma transfusion, and gamma globulin therapy and did not need hemodialysis. In the following months, she achieved clinical remission except for low complement C3. Since hemolytic uremic syndrome is rarely associated with IgA nephropathy, we present this case and discuss potential connection between hemolytic uremic syndrome and IgA nephropathy.