Cardiac tumors, either benign or malignant, are difficult to diagnose due to their rarity, variety, and nonspecific presentation. Since primary cardiac sarcoma remains an unusual diagnosis, the literature on its presentation, diagnosis, and optimal management remains scarce. To our knowledge the following case of cardiac perivascular epithelioid cell tumor is the fourth reported case found in the literature. Although complete surgical resection remains the gold standard for cardiac sarcomas, our case demonstrates that not all of them can be completely resected.