A 77-year-old man presented with Evans syndrome (ES), hard palate thickening, gastrointestinal (GI) hemorrhage, acute myocardial infarction (AMI) and pleural and pericardial effusions. The patient responded well to emergent ES treatment with high-dose steroids and intravenous immunoglobulin. Investigation revealed lymphoplasmacytic lymphoma (LPL) as well as amyloidosis in the hard palate, lymph nodes, and pericardium. Considering his age, non-myelosuppressive agents were administered, with the exception of dose-reduced cyclophosphamide. The patient developed neutropenic fever, atrial fibrillation and subsequently died. This report describes the first LPL patient with ES. LPL is generally an indolent disease. However, as in our patient, it can be life threatening because of its complications. ES contributed to his GI hemorrhage, severe anemia, and thus AMI at the time of presentation. Probable cardiac amyloidosis played a role in the latter phase (i.e. cardiac arrhythmia and hypotension during sepsis). Although rare, the presence of ES and amyloidosis should be investigated diligently in elderly LPL patients. Instead of aggressive myelosuppressive chemotherapy agents, targeted therapies might be considered in these fragile patients.