Purpose: To investigate clinical markers for the diagnosis of congenital cytomegalovirus (CMV) infection and determine the correlation between abnormal newborn hearing screening results and asymptomatic congenital CMV infection. Methods: Medical records of newborns with congenital CMV infection, born at Cheil General Hospital & Women's Healthcare Center from July 2008 to June 2018, were retrospectively reviewed. Infants with congenital CMV infection were classified into "symptomatic," "asymptomatic," and "asymptomatic with isolated abnormal automated auditory brainstem response (AABR)" groups. Clinical data were analyzed based on this classification. Results: Among the 59,424 live births, congenital CMV infection was found in 25 neonates, including 19 symptomatic (0.03%) infants, two asymptomatic, and four asymptomatic with isolated abnormal AABR. Diagnostic clues for the identification of congenital CMV infection were intrauterine growth restriction (IUGR), including microcephaly in 10 infants (40.0%), abnormal AABR in four (16.0%), initial complicated signs in four (16.0%), and abnormal findings on brain ultrasonography in three (12.0%). Other less common markers included petechiae, abnormal findings on antenatal ultrasonography, and co-twin with CMV infection. During the recent 10 years, 53,094 of 59,424 newborns (89.3%) had AABR for hearing screening and 493 (0.9%) did not pass. Among them, 477 (96.8%) were screened for CMV, and results were positive for seven (1.5%). Among the seven infants, four had asymptomatic congenital CMV infection. Overall, 0.8% of the newborns with abnormal AABR (four of 477 infants) were diagnosed as having asymptomatic congenital CMV infection. Conclusion: The incidence of symptomatic congenital CMV infection was 0.03%, and 0.8% of infants who failed in the newborn hearing screening tests had asymptomatic congenital CMV infection. The most common clinical marker to diagnose congenital CMV infection was IUGR, including microcephaly, and the second isolated marker was abnormal AABR.