Plasma cell leukemia (PCL) is an uncommon and aggressive plasma cell dyscrasia characterized by malignant plasma cells in bone marrow and peripheral blood. The prognosis of PCL patients treated with conventional chemotherapy remains poor. The clinical presentation of PCL features extensive abnormal plasma cells in the peripheral blood and a higher prevalence of organomegaly, with involvement of multiple tissues and organs, the symptoms usually include anemia, bleeding, infection, bone pain, and renal failure. However, PCL presenting as heart failure is very rare. We herein report a case of 45-year-old man primary PCL in which the patient presented atypically as heart failure.