Objective To explore the presentation and management of congenital cytomegalovirus (CMV) identifi ed through routine clinical investigations, and ascertain outcome in early childhood. Design Active population-based surveillance. Setting UK and Ireland. Methods Infants born in 2001-2002 with confi rmed or suspected congenital CMV infection were reported through the British Paediatric Surveillance Unit, and clinicians completed questionnaires on presentation, diagnosis, management and subsequent outcome. Results 86 confi rmed and 70 possible cases of congenital CMV infection were reported. Over a third (27/72) of singleton infants with confi rmed and 44% (27/61) with possible congenital infection were preterm (<37 weeks gestation). Among confi rmed cases, 75% (64/85) presented with neonatal manifestations compatible with congenital CMV, over half (34/64) of whom had neurological signs; 17 infants were treated with gancyclovir. Among confi rmed cases with information on outcome, 31% (24/78) were developing normally, 18% (14/78) had mild, 24% (19/78) moderate and 14% (11/78) severe sequelae, and 13% (10/78) had died. Median age at follow-up among survivors was 18 months (IQR 15-22 months). Children with neonatal CMV manifestations were signifi cantly more likely than those without to have moderate or severe outcomes (including death) (60%, 36/60, vs 22%, 4/18, p=0.001). 27% of survivors (17/63) had bilateral hearing loss. Conclusions The number of confi rmed cases of diagnosed congenital CMV reported in this study was lower than expected, highlighting the need for early and appropriate investigations when congenital infection is suspected. Due to the unexpectedly high proportion of preterm infants, resulting from differential case ascertainment, it was diffi cult to distinguish prematurity and CMV-related symptoms.