A case of Fraser syndrome diagnosed prenatally is presented. Detection of oligohydramnios, hydrops fetalis and bilateral absence of the kidneys were the initial findings leading to further study. Specific IgM for cytomegalovirus in maternal serum and confirmed infection by fetal blood sampling was an associated finding. The importance of an etiologic diagnosis of nonimmune hydrops and the relevant aspects of genetic counselling are emphasized. The association of the Fraser syndrome with cytomegalovirus infection has not been previously reported.