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Cardiac intimal sarcomas are extremely rare, remarkably aggressive and least reported type of primary malignant tumors of the heart. Cardiac intimal sarcomas are encountered more commonly in the large arterial blood vessels including pulmonary artery and aorta, and are extremely rare in the heart. The mainstay of treatment is achievement of tumor-free margins with surgical resection, which is associated with improved survival. Here, we report a 66-year-old female with primary cardiac intimal sarcoma presented with functional mitral stenosis and heart failure. This case report emphasizes common presentation of a rare disease, and the significance of early diagnosis and timely intervention.