Primary pericardial neoplasms are rare. These can be classified as benign and malignant. The pericardial mesothelioma is most frequent one in the malignant group. The clinical manifestations are variable and are presented as a consequence of the compression of the mediastinal structures, of which the most serious one is cardiac tamponade. Echocardiography is not a sensitive technique for the diagnosis of pericardial neoplasms. On computed tomography and magnetic resonance, pericardial mesothelioma appears as a mass of heterogeneous enhancement that involves both layers of the pericardium. The prognosis of this tumor is poor, with a survival rate of less than 15 months in most patients and frequently metastasizes to mediastinal lymph nodes and lungs.