In this case report, we present two Danish patients with hepatitis E virus (HEV) infection. In both cases, the route of transmission remained unclear. The infection led to typical symptoms in one case, whereas the other patient experienced neurological symptoms. Both patients recovered without treatment. This report provides a brief overview of our current knowledge of HEV infection and shows the importance of the infection as a differential diagnosis in patients, who present with elevated liver enzyme concentrations and extrahepatic symptoms despite lack of known relevant exposition.