Paragangliomas are rare neuroendocrine tumors that originate in the chromaffin cells of the adrenal medulla or lymph nodes. Paragangliomas manifest in rare cases as catecholamine crisis, leading to heart failure, intracranial hemorrhage, renal failure, arrhythmias, pulmonary edema, or multisystem failure. Takotsubo cardiomyopathy is also called apical ballooning syndrome or stress cardiomyopathy. Left ventricular dysfunction with apical hyperkinesis and basilar and midventricular akinesis in the absence of coronary artery disease is highly suggestive of a variant of stress cardiomyopathy (inverted takotsubo cardiomyopathy). Herein, we report the case of a 69-year-old man with an unknown retroperitoneal paraganglioma who suffered from cardiogenic shock due to inverted takotsubo cardiomyopathy. He was treated with venoarterial extracorporeal membrane pulmonary oxygenation (ECMO) in combination with an intra-aortic balloon pump. After the restoration of cardiac function, a successful transition to curative retroperitoneal paraganglioma resection was performed. We conclude that ECMO is a valuable option for undiagnosed endocrine emergencies, helping to restore cardiac function and allowing sufficient time for further accurate diagnosis and specific treatment.