Congenital cytomegalovirus (CMV) infection is one of the most common viral causes of congenital infections in high resource countries and a leading cause of hearing loss as well as an important contributor to neurodevelopmental disabilities in children. During early pregnancy, CMV has a teratogenic potential and may cause malformations such as migrational disturbances in the brain, which can be visualised using neuroimaging methods such as magnetic resonance imaging (MRI) in such children. As a consequence of variation in epidemiology and seropositivity in fertile women, the prevalence of congenital CMV in their offspring varies in different countries between 0.15-2.0%. Some 10-20% of all children with congenital CMV infections exhibit signs of neurological damage when followed up. This is the case in children both with and without symptoms of infection at birth. Until vaccines and non-toxic antiviral agents are available, hygienic measures are important as prophylaxis. Treatment with antiviral agents may have a place in children with central nervous system involvement during the neonatal period.