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Pericardial effusion is usually caused by infection, fluid overload states, connective tissue disorders, heart surgery, aortic dissection, and malignancy. When a patient presents with recurrent isolated pericardial effusion accompanied by a nonspecific history and negative laboratory tests, it can pose a diagnostic dilemma to the clinician. Primary malignant tumors of the pericardium are sporadic, and most primary malignant pericardial tumors are mesotheliomas. We report the case of a young adult male with recurrent pericardial effusion and no specific clinical clues enabling an early diagnosis, which later turned out to be caused by a primary angiosarcoma of the pericardium.