Acute disseminated encephalomyelitis (ADEM) is an acute monophasic inflammatory and demyelinating disease of the central nervous system (CNS) occurring days to weeks after a virus infection or vaccination. Nephropathia epidemica (NE) is a haemorrhagic fever with renal syndrome caused by Puumala virus, with endemic regions in Europe, especially Scandinavia and Western Russia. We describe a case of severe nephropathia epidemica requiring dialysis, followed by severe CNS symptoms caused by ADEM. To our best knowledge this is the first case in the literature in which NE caused ADEM.