Cytomegalovirus (CMV) is the leading cause of congenital infection and the most common cause of non-genetic sensorineural hearing loss (SNHL) in childhood. Although most infected infants are asymptomatic at birth, the risk for SNHL and other neurodevelopmental morbidity makes congenital CMV (cCMV) a disease of significance. Adherence to hygienic measures in pregnancy can reduce risk for maternal CMV infection. The prompt identification of infected infants allows early initiation of surveillance and management. A multidisciplinary approach to management is critical to optimize outcomes in affected infants.