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Primary cardiac lymphoma is a very rare malignancy, which is typically of a non-Hodgkin type, and involves only the heart and pericardium with no or minimal evidence of extracardiac involvement. Primary cardiac lymphoma account for about 1% of the primary cardiac tumors and 0.5% of the extranodal lymphomas. On the other hand, disseminated lymphoma with cardiac involvement can occur in up to 20% of patients with lymphoma. About 80% of cases of the primary cardiac lymphoma in immunocompetent hosts are of diffuse B-cell lymphoma, and in patients with immunodeficiency states, small noncleaved or immunoblastic lymphomas are more frequent. The right atrium and right ventricle are the 2 most frequently involved sites. Clinical presentation is heterogeneous and is generally related to the site of involvement in the heart. The diagnosis is suspected when patients present with a cardiac mass or an unexplained refractory pericardial effusion. A thorough workup should include transthoracic and transesophageal echocardiography, computed tomography, and magnetic resonance imaging. Diagnosis is confirmed by cytology of the serous fluid from pericardial or pleural effusion or biopsy of the pericardial mass or endomyocardial tissue. The exploratory thoracotomy should not be delayed if indicated. Chemotherapy has been used alone or combined with radiotherapy. Similarly, palliative cardiac surgery has been performed, mainly for tumor debulking. Combination of chemotherapy and radiation therapy is considered as the treatment of choice. The survival is generally less than a month without treatment but has been prolonged up to 5 years with palliative treatments in selected cases.