Background: Myxomas cause about 0.5% of strokes. Cardiac sarcomas are rare, may mimic myxomas, and are also associated with stroke. Imaging can provide clues to diagnosis. Case: A 53-year-old woman with diabetes, hypertension and prior strokes was admitted with new left hemiparesis. Computed tomography (CT) of the head revealed large middle cerebral artery infarct with petechial hemorrhages. Transthoracic echocardiogram (TTE; panel A) revealed large left atrial (LA) mass suspicious for myxoma protruding through the mitral valve. CT of the chest (panel B) revealed hilar adenopathy and LA mass concerning for myxoma or thrombus with stalk attachment to the right pulmonary vein (PV). Decision-making: Following heart team meeting decision was to remove the mass surgically given its embolic potential. Intraoperative transesophageal echocardiogram (TEE; panel C) revealed a large LA mass with multiple papillary protrusions and attachment via a stalk to the right lower PV. The mass was successfully removed and the patient recovered and was discharged. Follow up pathology returned as cardiac sarcoma. Conclusion: Pulmonary vein intimal sarcomas are rare tumors with poor prognosis, presenting early with chest pain, dyspnea and hemoptysis, or late with stroke. They can mimic myxomas and are therefore often diagnosed post-operatively. Preoperative diagnosis however is imperative to plan treatment and for prognostication. TEE and cardiac magnetic resonance imaging are the modalities of choice to diagnose sarcomas and evaluate extent of cardiac structure involvement.