A cardiac tumor was the first manifestation of acquired immunodeficiency syndrome (AIDS) in a female patient in a state of severe immunodeficiency caused by human immunodeficiency virus (HIV) infection. The extensive cardiac and extracardiac involvement shown by various imaging modalities, including echocardiography and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET), suggested that she was in the critical stage of non-Hodgkin's lymphoma (NHL). AIDS was treated by highly active-antiretroviral therapy and the NHL was treated by a combination of rituximab-cyclophosphamide-vincristine-doxorubicine-predonisolone. After 6 cycles of chemotherapy, she was in complete remission. Her cardiac tumor dramatically reduced in size and FDG-PET showed no positive uptake on whole body imaging. Generally, an AIDS-related cardiac tumor tends to be diagnosed at the late stage of the disease because of its nonspecific clinical findings, resulting in an extremely poor prognosis. In the present case, the cardiac tumor was detected by echocardiography and treated with appropriate chemotherapy. Early diagnosis and prompt treatment may improve a patient's prognosis.