We present a case of malignant angioendotheliomatosis (MAE) localized at the central nervous system (CNS) in a 74-years-old male, manifested as an acute confusional syndrome with fever, characteristics of lymphocytic meningitis in the cephalorhachidian liquid (CRL) and dilatation of the left lateral ventricle according to the cranial computerized tomography (CT). The diagnosis was established using post-mortem immunohistological techniques, concluding that it was an intravascular lymphomatous process with secretion of immunoglobulins.