AbstractHuman cytomegalovirus is worldwide the most common pathogen causing teratogenic congenital infection. Maternal cytomegalovirus (CMV) infection is a serious health concern as it is associated with severe birth defects like sensori-neural hearing loss, impairment of cognitive development and cerebral palsy. It is acquired by contact with body fluids and by vertical transmission from mother to fetus. Transmission rates are particularly high after primary infection of women. Unlike rubella infection, past infection with CMV and seropositivity at the time of conception does not confer protection to the fetus. The rate of transmission increases with period of gestation but the severity is more when an infection is transmitted to fetus before 20 weeks gestation. Congenital CMV (cCMV) infection may present as cerebral or extracerebral ultrasound abnormalities, fetal growth restriction and fetal loss. Hearing loss is the most common long term sequelae of cCMV infection accounting for about 25–30% of all cases of hearing loss in children in the US. Immunoglobulin G avidity testing is used to better define the timing of the infection in the mother. Invasive testing and ultrasound aid in the diagnosis of fetal infection. Hygiene information to pregnant women for prevention of CMV infection is currently the most effective preventive strategy. The role of vaccines, antiviral drugs and immunoglobulins is still not proven.