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Idiopathic hypereosinophilic syndrome (IHES) is a rare systemic disease with multiple clinical presentations including cardiac, pulmonary and dermatologic manifestations. Little is known about the pathophysiology and potential triggers of this condition. Approximately 40–70% of these cases have cardiac involvement, which is the major cause of morbidity and mortality. Cardiac tamponade is a rare but serious complication of IHES. Herein, we report a patient with IHES who presented with cardiac tamponade. To the authors' knowledge, this is the second case of cardiac tamponade in adults with IHES reported in the medical literature.