Abstract: Evans syndrome is defined by the simultaneous or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia, which is classified as primary or secondary. Hematological malignancy can be a common secondary underlying disease. Here we report a case presenting as primary Evans syndrome after excluding all possible secondary disorders. Evans syndrome improved after treatment of rituximab combined with bortezomib. One year later, the symptoms relapsed and small B-cell lymphocytic lymphoma was eventually diagnosed. The recurrence of disease, laboratory examinations, diagnostic process, and treatment were analyzed and discussed not only to remind clinicians that secondary factors should be re-evaluated in recurrent or refractory Evans syndrome, but also to provide help for clinicians to diagnose and treat such diseases. It is often necessary to remove the secondary factors in secondary Evans syndrome so that the disease can be effectively controlled.