Study Design. Case report and review of the literature. Objective. To present a case with an uncommon nonfunctioning malignant paraganglioma of the posterior mediastinum with compression of the spinal cord. An update survey is given with respect to diagnostic, histopathologic, and therapeutic aspects of paragangliomas. Summary of Background Data. Paragangliomas in the posterior mediastinum are uncommon and rarely may produce spinal cord compression. To illustrate the low incidence, only two cases of paraganglioma of the posterior mediastinum were reported by the Mayo Clinic, Rochester, Minnesota, during a 40-year period. Method. Case report and review of the literature. Result. The diagnosis was made by modern imaging techniques (computed tomography, magnetic resonance imaging, and metaiodobenzylguanidine scan) and verified by computed tomography guided per thoracic puncture. A wide local resection was performed; the patient is disease-free 1 year after surgery. Conclusion. The uncertainty of prognosis and possibility of local recurrence of paragangliomas even after a long period emphasizes the importance of wide local surgical resection with or without adjuvant therapy and makes long-term follow-up and continued surveillance of the patient mandatory.