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Primary testicular lymphoma (PTL) is a rare but aggressive disease. Although most patients present in the early stage, their prognosis is poor. Similar with PTL, cardiac lymphoma is also an uncommon disease characterized by its aggressive clinical course and poor prognosis. We herein report an extremely rare case of advanced stage PTL with cardiac involvement, treated by high-dose chemotherapy with autologous stem cell transplantation (HDT-ASCT) followed by systemic chemotherapy, prophylactic intrathecal methotrexate (IT-MTX), and radiotherapy. A 48-year-old man presented with painless left scrotal swelling. He was diagnosed with PTL after orchiectomy, and the histological type was diffuse large B-cell lymphoma. For staging of lymphoma, positron emission tomography was performed, which revealed uptake in the right atrium and early cardiac metastasis within just 2 months after orchiectomy. He underwent 6 cycles of systemic chemotherapy that consisted of rituximab, doxorubicin, cyclophosphamide, vincristine, and prednisolone (R-CHOP). He also received central nervous system prophylaxis 4 times with weekly IT-MTX during the first 2 cycles of R-CHOP. He achieved complete response after 6 cycles of R-CHOP, and underwent HDT-ASCT and radiotherapy as consolidation therapy without irreversible adverse effects. He is currently doing well, with a progression-free survival of 31 months. The above treatment strategy including HDT-ASCT may be one of the treatment options for advanced stage PTL with cardiac metastasis in patients younger than 65 years old.