Sudden cardiac death is an unexpected clinical condition that typically occurs due to a cardiac cause, generally within 1 h of symptom onset, in people with known or unknown cardiac disease. Primary malignant pericardial mesothelioma, as a cause of sudden death, is an uncommon consequence of a rare disease. Herein, we present a case of cardiac tamponade due to a primary pericardial mesothelioma. Cytological, histopathology and gross post-mortem findings, in a previously asymptomatic 46-old-year man, are reported. The medical literature regarding this topic is also reviewed.