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We report a case of pheochromocytoma presenting with a sudden change in clinical manifestation. The change in the serum Interleukin-6 (IL-6) and histological examination indicated that this clinical presentation was caused by IL-6 elevation secondary to pheochromocytoma. This rare tumor should be considered in the differential diagnosis of fever of unknown origin, and prompt and appropriate administration of an alpha-1 blocker is highly recommended, especially for patients with large pheochromocytomas.