We describe the case of a patient who presented with a kidney lesion, lymphadenopathy, and bone metastasis. He developed a tetrad of high fever, encephalopathy, labile blood pressure, and multiple organ system failure. Initially, he was diagnosed with metastatic renal cell carcinoma (RCC). His fevers and encephalopathy were compatible with a putative paraneoplastic syndrome. Eventually, he was found to have malignant pheochromocytoma. To our knowledge, this is the first reported case of a malignant pheochromocytoma with multisystem crisis. Because of its atypical presentation and unusual findings, a high index of suspicion is necessary for the timely diagnosis of this clinical entity. Prompt diagnosis and appropriate treatments could improve the performance status and quality of life of such patients.