Metastatic disease in the intradural compartment of the spine is a rare manifestation of cancer. We report the case of an 82-year-old patient with an intradural, extramedullary metastasis of renal cell carcinoma in the cervical spine. A literature search for intradural spinal metastases of renal cell carcinoma yielded a total of 26 further cases. 18 patients had sporadic renal cell carcinoma, and 9 patients had von Hippel-Lindau disease (VHL) in which the metastases of the renal cell carcinoma were embedded within spinal haemangioblastomas. Patients presented with paresis, back pain, altered sensation or, less frequently, bladder dysfunction. Intradural spinal metastases were diagnosed at an earlier age in VHL patients than in sporadic cases (mean 43 +/- 5 years vs. 60 +/- 14.5 years). The metastasis was surgically removed in 81% of patients. Pain improved in all patients, paresis in 90%, hypaesthesia in 38% and bladder dysfunction in 50%. Death occurred as a result of systemic cancer progression. 93% of patients in the sporadic renal cell cancer group died within 1.5 years, whereas two thirds of the VHL patients were alive after 2 years.