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Primary testicular lymphoma (PTL) is a rare testicular tumor representing less than 9% of all testicular cancers. PTL usually tends to spread to or relapse at nodal structures or extra-nodal sites such as contralateral testes, central nervous system, skin, lung, pleura, waldeyer’s ring and soft tissues. We present a case of PTL with huge left atrial mass, an extremely unusual site of involvement. Early disease usually carries a good prognosis, whereas advanced stage carries an extremely poor prognosis. Herein, we report the complete remission to date in a patient with advanced stage PTL with huge left atrial mass, treated with systemic rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone and intrathecal methotrexate. A brief review of literature focusing on various aspects of management of primary testicular lymphoma and lymphomatous involvement of heart is also discussed

Primary Testicular Lymphoma with Cardiac Involvement in an Immunocompetent Patient: Case Report and a Concise Review of Literature