BACKGROUND AND OBJECTIVES: Congenital cytomegalovirus (CMV) is the leading cause of nongenetic deafness in children in the United States and can cause neurodevelopmental impairment in term infants. Limited data exist regarding congenital CMV infections in preterm infants. We aimed to determine the incidence and association with outcomes of congenital CMV in very low birth weight (VLBW) preterm infants. METHODS: VLBW infants born in 1993 to 2008 and admitted to the University of Alabama in Birmingham Regional Neonatal ICU were screened on admission for congenital CMV. CMV status and clinical outcomes were identified by using internal patient databases and hospital-based medical records. The primary outcome was death. Secondary outcomes included evidence of neurologic injury in the form of abnormal cranial ultrasound findings, sensorineural hearing loss, or abnormal motor development. Multivariate analysis was performed. RESULTS: Eighteen of 4594 VLBW infants had congenital CMV (0.39%; 95% confidence interval, 0.25%-0.62%). An additional 16 infants (0.35%; 95% confidence interval, 0.21%-0.57%) were identified who acquired CMV postnatally. Congenital CMV was not associated with death. Compared with controls, congenitally infected VLBW infants were more likely to have hearing loss at initial screening (67% vs 9%, P < .0001) and confirmed at follow-up (83% vs 2.1%, P < .0001). Congenital CMV was also associated with abnormal neuroimaging (72% vs 25%, P < .0001) and adverse developmental motor outcomes (43% vs 9%, P = .02). Acquired CMV was not associated with any adverse outcomes. CONCLUSIONS: Congenital CMV in VLBW infants is associated with high rates of neurologic injury and hearing loss but not death. Copyright © 2014 by the American Academy of Pediatrics.