We report a case of fatal congenital cytomegalovirus (CMV) disease in a 695gm, 29 weeks estimated gestational age premature infant. The newborn presented with hydrops fetalis, an unusual presentation of congenital CMV infection. In spite of ganciclovir therapy, the infant succumbed to his illness. Autopsy findings revealed the presence of widespread CMV disease, including pneumonitis, enteritis, and myocarditis. Congenital CMV infection should be considered in the differential diagnosis of hydrops fetalis.