Primary cardiac lymphoma is an extremely rare malignancy, which is typically of a non-Hodgkin's type. Clinical manifestations including dyspnea, arrhythmia, chest pain and so on, may vary according to heart site involved and the right atrium is most frequently involved. Histologically, the majority of primary cardiac lymphomas are B-cell origin, especially diffuse large B-cell lymphoma. Here we report a case of primary cardiac nature killer/T cell lymphoma arising in a 40-year-old woman, who presented with paroxysmal chest congestion, nausea and ventricular tachycardia, and diagnosed by open chest surgery and whole body PET/CT scan, then treated with three cycles of chemotherapy, but finally died of multiple organ failure. The optimal treatment strategies to primary cardiac lymphoma have not been fully defined due to its rarity, which still needs to gather more cases and conduct further researches.