The authors report the case of a 70-year-old woman who developed a Brown-Sequard-syndrome within 6 weeks caused by an intramedullary spinal cord metastasis of an occult renal cell carcinoma. Intramedullary metastases are rare and represent only 4-8.5% of central nervous system metastases. An important feature of intramedullary metastases is the rapid progression of neurological deficits which necessitates rapid treatment. There are only eight earlier reports of intramedullary metastasis due to renal cell carcinoma (Schiff D, O'Neill BP. Intramedullary spinal cord metastases: clinical features and treatment outcome. Neurology 1996;47:906-12; Belz P. Ein Fall von intramedullaerer Grawitz-Metastase im Lumbalmark. Frankfurt Z Pathol 1912;10:431-44; Gaylor JB, Howie JW. Brown-Sequard-syndrome. A case of unusual aetiology. J Neurol Neurosurg Psychiatry 1938;1:301-5; Kawakami Y, Mair WGP. Haematomyelia due to secondary renal carcinoma. Acta Neuro Pathol 1973;26:85-92; Strang RR. Metastatic tumor of the cervical spinal cord. Med J Aust 1962;1:205-6; Von Pfungen. Uber einige Fälle von Haematomyelie nichttraumatischen Ursprungs. Wien Klin Rdsch 1906;20:44-50; Weitzner S. Coexistent intramedullary metastasis and syringomyelia of cervical spinal cord. Report of a case. Neurology 1960;674-8). To the best of our knowledge this is the first report on a patient in whom symptoms from the metastasis of a renal cell carcinoma preceded the detection of the primary tumor. This report presents the clinical, neuroradiological and histopathological findings of an intramedullary metastasis of a renal cell carcinoma and provides an overview of the literature on intramedullary spinal cord metastases.