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A 35 year old gentleman presented to a medical centre with backache and palpitation for a month. He was referred to our centre for right atrium(RA) mass. He underwent open heart surgery via median sternotomy. The large tumour was raised from interatrial septum, extending into the IVC and the postero-inferior part of RA. The inferior vena cava (IVC) was fixed posteriorly due to the tumour invasion. Debulking surgery was performed. Histopathology and immunohistochemical study confirmed a malignant hemangioendothelioma. He has completed a palliative course of tomotherapy. He is treating with Tazemetostat, an inhibitor of EZH2 methyltransferase. He remains asymptomatic from cardiac aspect after 1 year of surgery.