Cardiac angiosarcoma is a rare and clinically challenging pathology. It is a high-grade primary malignant tumor of the heart tissue that has many variants, of which the epithelioid variant is rarely present in the heart or the great vessels. As with many other cardiac tumors, it is mainly a diagnosis of exclusion and the initial diagnostic test is an echocardiogram followed by a biopsy with immunohistochemistry analysis to ascertain the type of tumor. The differential diagnosis of cardiac tumors is challenging due to the overlapping clinical manifestations with different cardiac tumors and systemic diseases. Cardiac angiosarcomas are often aggressive with a poor prognosis even with treatment. Herein, we present a case of the epithelioid variant of a cardiac angiosarcoma in addition to a thorough review of the recent literature on the clinical manifestation, diagnosis, and treatment of this type of tumors.