Cardiac sarcoma is the commonest histology among primary cardiac tumors but it is a rare clinical entity and it is characterized by late stage presentation, poor prognosis from the time of detection and a variety of clinical presentations depending on the size and extent of the mass. We report the case of a high-grade right atrial angiosarcoma presenting with pulmonary embolism and cardiac tamponade. The patient underwent surgical resection and reconstruction of the atrial wall with bovine pericardial patch. This case report underscores the great heterogeneity of the clinical presentation, often non-specific, and the fatal prognosis of angiosarcoma, mostly related to the lack of evidence supporting a standardized therapeutic approach.