Autochthonous hepatitis E virus (HEV) infection has been increasingly reported in Europe and the United States, mostly arising from genotype 3 and less frequently genotype 4. We report here on a patient with HEV genotype 3a infection complicated by Guillain-Barré syndrome in Portugal in December 2012. We draw attention to the diagnosis of autochthonous HEV infection and to its rare, but important, neurological complications.