Intravascular malignant lymphomatosis (IML) is characterised by proliferation of tumoral cells within small vessels. Angiotropic large cell lymphoma commonly affects the central nervous system and the skin, although any organ may be involved. Few cases are diagnosed ante mortem. We report a new case of IML in a 62-year-old woman who presented with the main clinical features of central nervous system involvement: cerebrovascular events, epileptic seizures, subacute dementia and spinal cord syndrome. Cranial magnetic resonance imaging demonstrated increased periventricular signal on T2-weighted images. Diagnosis was established by brain biopsy. Despite corticosteroid and radiation therapy no improvement occurred and the patient died 14 months after the onset of the first symptoms.