A rare case of a paraganglioma of the filum terminale is reported. The pati
ent presented with headache and papilledema, Magnetic resonance imaging dem
onstrated enlarged ventricles of the brain and a well-demarcated intradural
spine tumor. After resection of the tumor, the papilledema and headaches r
esolved, 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 associat
ion of spinal tumors with hydrocephalus.