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The following manuscript documents the management of a middle-aged male, who underwent surgical resection of a large, right atrial cardiac lipoma. The patient presented with an extensive oncologic history including osteogenic sarcoma, melanoma and pulmonary neoplasm, suggesting that his cardiac mass was part of a syndromic presentation. In this case, surgical resection was pursued both to treat his increased symptom burden and to obtain definitive tissue diagnosis. Hence, this case report demonstrates how a syndromic presentation of cardiac lipoma requires a lower threshold for surgical resection, especially when considering heightened malignancy risk.