The spinal cord is an extremely rare site for primary central nervous system (CNS) lymphoma (< 1%). Very few cases of primary cauda equina (including conus) lymphoma were previously reported. We report such a case, and with literature review, discuss their clinical features, operative and histopathological findings. Although rare, with an increasing incidence of CNS lymphoma, they should be considered in the differential diagnosis of intradural lesions. Furthermore, with intraoperative smear to establish diagnosis, extensive surgery can be avoided. The controversial role of glucocorticoids in the management of these patients is also discussed.