Congenital CMV is a major cause of neurological and sensory impairment in children. Reliable estimates of the prevalence of permanent sequelae and mortality associated with congenital CMV are needed to guide development of education and prevention programmes and to gauge the financial costs associated with this disease. To calculate such estimates, this review used data solely from studies in which children with congenital CMV were identified through universal screening. Based on 15 studies with a total of 117 986 infants screened, the overall CMV birth prevalence estimate was 0.7%. The percentage of infected children with CMV-specific symptoms at birth was 12.7%. The percentage of symptomatic children with permanent sequelae was 40-58%. The percentage of children without symptoms at birth who developed permanent sequelae was estimated to be 13.5%. The true burden of congenital CMV infection is unclear because data on important outcomes, such as visual impairment, are lacking and follow-up of infected children has been too short to fully identify late-onset sequelae. Therefore, the estimates of permanent sequelae associated with congenital CMV presented here are likely underestimates. Future studies should extend follow-up of CMV-infected children identified through universal screening and include the evaluation of visual impairment.