Neonates infected with nonpolio enteroviruses are at high risk for developing significant illness, including sepsis-like illness, meningoencephalitis, myocarditis and/or hepatitis. Echoviruses and group B coxsackieviruses account for the majority of neonatal enterovirus infections. We reported a case of echovirus 11 infection in newborn associated with maternal infection. To our knowledge, this is the first reported fatal case of neonatal echovirus infection in Taiwan. Eventually, the baby expired because of severe sepsis-like illness, fulminant hepatitis, disseminated intravascular coagulation, and extensive hemorrhagic manifestations in spite of intensive care, intravenous immunoglobulin infusion and exchange transfusion.