Autoimmune hemolytic anemia (AIHA) is an unusual complication of malignancy. We diagnosed primary splenic anaplastic large cell lymphoma (ALCL) in a patient. A seven-year-old boy presented with Coombs test-positive hemolytic anemia. After a course of prednisolone therapy, a complete response for anemia was achieved. Twenty months later, in addition to severe hemolytic anemia, the patient was diagnosed with ALCL after splenectomy and pathologic examination of the sample. The recognition of this clinical picture as a complication of non-Hodgkin's lymphoma has important implications. The most effective management of AIHA in the setting of cancer is to treat the underlying malignancy.