A rare case of a paraganglioma of the filum terminale is reported. The patient presented with headache and papilledema. Magnetic resonance imaging demonstrated enlarged ventricles of the brain and a well-demarcated intradural spine tumor. After resection of the tumor, the papilledema and headaches resolved, and the ventricles returned to normal size. Pathologic examination revealed a paraganglioma of the filum terminale. We present this rare case and review the literature of spinal paragangliomas as well as the association of spinal tumors with hydrocephalus.