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Primary cardiac lymphoma (PCL) is a rare extranodal lymphoma involving only the heart and/or the pericardium. Most common presenting signs and symptoms are nonspecific including dyspnea, pericardial effusion, and arrhythmia. Prognosis of PCL patients remain poor compared to non‐cardiac lymphoma patients. Since most of the information about PCL comes from case reports or case series, there is no treatment consensus. Anthracycline containing chemotherapy remains main treatment modality which is potentially cardiotoxic. We present a case of PCL that achieved complete remission using R-EPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin). We also used dexrazoxane in an effort to reduce cardiotoxicity of chemotherapy.