Background: Paragangliomas affecting the filum terminale are extremely rare, benign tumors. The literature yielded thirty-two cases of paraganglioma in this site. Case presentation: A 49 year-old-man, whose presenting symptoms were low back pain and left leg weakness, was diagnosed as having a paraganglioma of the filum terminale. The clinical, histological and radiological characteristics of this case, that brings the total number of cases described to 33, are discussed in the light of published data. Conclusions: This extremely rare pathology can usually be successfully treated by total surgical resection, which represents the gold standard. In the event of incomplete removal, assiduous long-term follow-up is mandatory.
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