Although rare, cauda equina paraganglioma (CEP) is definitely established in the table of cauda equina tumours. There are no particular investigations to document its preoperative diagnosis, unless a vasomotor amine syndrome is present to guide the differential diagnostic spectrum. Magnetic resonance imaging is the examination of choice for the presurgical assessment, but there are still overlapping findings, mainly among CEP, ependymoma, schwannoma, and hemangioblastoma. It is also the only means for postoperative follow-up. Computed tomography may fail to reveal all intrathecal lesions. Total excision is the gold standard for lesions with such a benign or at least protracted clinical course, as observed with the CEP. In contrast to well-known oncologic principles in micro, ultrastructural, and immunohistochemical prediction analysis, these tumours behave in an independent fashion. Promising efforts are being conducted in immunohistochemistry and DNA search, but the mainstay of our knowledge regarding its natural history still stems from our sporadic observation of its clinical behaviour.