A case of malignant pheochromocytoma arising from the abdominal preaortic chromaffin tissue is presented. Of particular note is that right hemiplegia associated with sensory disturbances, compatible with the Brown-Séquard syndrome, was an outstanding early clinical manifestation. The patient finally developed signs of complete transection of the spinal cord which was caused by metastasis of the tumor to the cervical spines. The neurological abnormalities observed in the present case were so unusual in pheochromocytoma as to lead initially a diagnosis of cord tumor. Autopsy findings well supported the clinical diagnosis. Cases like this have not been reported so far.