Evans syndrome is a rare syndrome associated with the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and idiopathic thrombocytopenic purpura (ITP). Furthermore, acute kidney injury (AKI) is a syndrome characterized by the rapid loss of kidney excretory function and is most often secondary to extrarenal events. However, AKI has rarely been recorded in Evans syndrome without systemic autoimmune disease and malignant tumors of the blood and lymphatic system. Herein, we report the case of a patient who exhibited Evans syndrome presenting with AKI. A 73-year-old woman presented with diarrhea, anuria, low platelet count, and developed a progressive increase of blood urea nitrogen and serum creatinine, as well as anemia with a positive direct Coombs test. We excluded hemolytic uremic syndrome, ITP, and leukemia. Treatment with antibiotics, rehydration therapy, and hemodialysis resulted in partial remission; thus, we diagnosed the patient with Evans syndrome presenting with AKI. The patient was successfully treated by the addition of steroid treatment. When AKI presents with hemolysis and thrombocytopenia, physicians should consider Evans syndrome, which can be appropriately treated when detected early.

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