A congenital cytomegalovirus (CMV) infection in a quadruplet has not been reported previously in the literature. We describe a congenital CMV infection in a quadruplet after primary CMV infection of the mother during pregnancy. One infant died antenatally, while another had cholestatic jaundice at birth and died of liver failure at three months of age. Of the two surviving infants, one showed no signs or symptoms of congenital CMV infection at 18 months of age, whereas the other had hearing loss and delayed development. CMV cultures of urine were positive at six weeks of age in all three infants born alive. The diagnosis of congenital CMV infection after primary CMV infection of the mother during pregnancy was made retrospectively by detection of CMV-immediate early antigen in three placentas as well as by examination of serum obtained from the mother during pregnancy.