Abstract Objective Congenital cytomegalovirus (CMV) infection is a common intrauterine infection and the leading cause of nonhereditary sensorineural hearing loss. This study aims to assess the long-term outcome of the infection and to identify infants at risk of developing long-term sequelae. Methods This retrospective single-center observational study includes infants born between 2003 and 2019 with confirmed congenital CMV (based on current criteria). Brain imaging (ultrasound and magnetic resonance imaging [MRI]), clinical monitoring of neurosensory development, and auditory brainstem evoked responses were performed, as well as long-term neurodevelopmental follow-up to assess sequelae. Results A total of 66 infants with congenital CMV were included in the study. Median gestational age at birth was 38.6 weeks (interquartile range: 36.9–40.1). Clinical findings included intrauterine growth restriction (39%), microcephaly (29%), thrombocytopenia (17%), and jaundice (11%). Brain abnormalities were observed on ultrasound (30%) and MRI (42%). Neurodevelopmental scores were abnormal in 21 subjects (43%) and associate with the vacuolization of anterior temporal lobe and ventricular septations on MRI (both p = 0.05). Fourteen patients (21%) had sensorineural hearing loss, which was more common in patients with abnormal cerebral images at birth, determined by ultrasound (p = 0.06). Microcephaly (p = 0.05) and abnormal MRI (p = 0.03) at birth were associated with poor long-term outcomes. Conclusion Early detection of congenital CMV infection is important to prevent long-term complications in affected infants. Understanding the predictors of poor outcomes may help improving management and treatment strategies for this condition.