Primary cardiac sarcomas are malignant neoplasmas of mesenchymal cells. Angiosarcomas, unlike all other primary cardiac sarcomas, are typically found in the right atrium. We present the case of a 52-year-old patient in whom through echocardiography, computed tomography and angiography the precise diagnosis and extent of the tumor was revealed. Surgical treatment consisted of excision of the tumor with right atrium patch-plasty under cardiopulmonary bypass. Despite the successful operation the patient died 6 months later because of metastasis. Very early and precise diagnosis is essential to improve patient survival and prevent the development of metastases.