Congenital cytomegalovirus (CMV) infection is the most common congenital viral infection and can be a major cause of neurodevelopmental disabilities, including various ocular disorders in infants and young children. This review summarizes the evidence on the association between congenital CMV infection and the type and frequency of ocular disorders. A systematic search was conducted across PubMed and Cochrane Library from inception through December 2021 to identify studies examining the association between congenital CMV infection and the occurrence of ocular disorders in children born with this infection. Seventeen articles were identified. A total of 306 ocular disorders were identified in 977 children, with 45 of them (4.6%) being related to cases of chorioretinitis. A total of 259 of the 306 (84.6%) ocular disorders occurred in symptomatic children. Four studies did not report the number of children with ophthalmological disorders. The remaining 13 studies reporting this parameter included 666 children, of whom 85 had ophthalmological disorders (12.7%). Vision was tested in 556 children and visual impairement was detected in 91 of them (16.3%). Given the high prevalence of congenital CMV infection and the frequent occurrence of ocular disorders, the question that arises is whether there should be worldwide screening for congenital CMV infection so that both symptomatic and asymptomatic children can be detected in time to reduce the burden of the disease and its complications. [J Pediatr Ophthalmol Strabismus. 2023;60(2):86-94.].