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Paraganglioma is an extraadrenal pheochromocytoma originating from chromaffin cells distributed in the sympathetic nervous systems. Functioning extraadrenal paragangliomas represent more than 10% of all pheochromocytomas, and seems to be highly malignant tumor in comparison to intraadrenal pheochromocytomas. Recently, we experienced a case of a paraganglioma in the posterior mediastinum. A 32-year-old woman was admitted to hospital due to dyspnea on exertion, and intractable hypertension. A chest X-ray showed a well-defined mass density on the right cardiac border, and biochemical studies showed characteristic findings of pheochromocytoma. A solitary pheochromocytoma was located in the posterior mediastinum using 131I-MIBG scintigraphy. The clinical manifestations, including hypertension and dyspnea were improved after operation.