Intravascular malignant lymphomatosis (IML), so-called malignant angioendotheliomatosis, was found in lumbosacral spinal cord and nerve roots of a 78-year-old women who died one month after the onset of symptoms. With regard to the majority of the 37 reviewed neurological cases in the literature, this report is unusual in that the disease was exclusively localized in the spinal cord and systemic involvement was absent. The usual clinical hallmark of the disease is a subacute dementia or encephalopathy, often associated with focal neurological signs, culminating in death within several months. The pathological features of IML characteristically include multiple small foci of necrosis of the whole brain, caused by occlusion of small vessels by noncohesive neoplastic cells and secondary changes of the vascular wall. All organs may be involved, especially the skin and the adrenals, sometimes with tumoral formations. Despite the fact that lymphoid tissues are usually spared, recent reports and the present case strongly suggest a lymphoid rather than endothelial origin of the malignant cells. The pathogenesis of this mainly intravascular lymphoma remains obscure.