Pericardial mesothelioma is a highly lethal and fortunately rare cardiac neoplasm. We present the clinical and pathologic features of a primary sarcomatoid mesothelioma. To better understand the clinical, radiographic, and pathologic features of this entity, we reviewed 27 cases described in the English literature from 1972 through 1992, which, together with our case, provided a total of 28 cases. Findings of the review include a male-female ratio of 2:1, a wide age range (12 to 77 years; mean, 47 years), and documented asbestos exposure in 4 of 28 (14%) patients. Commonly used imaging studies do not appear to offer great sensitivity, for a mass was detected by echocardiography in only 2 of 16 (12%) patients and by computed tomography in 4 of 9 (44%). Pathologic findings revealed a diffuse growth pattern in most cases (18 of 25, or 72%), together with an equal distribution between the biphasic, epithelioid, and sarcomatoid variants. Effusion cytology revealed malignant cells in only 2 of 10 (20%) cases. With or without therapy, prognosis was uniformly poor, since 24 of 27 patients were dead of the disease at the time the reports were published.