The recent Zika virus (ZIKV) outbreak and the link to birth defects in newborns exposed in utero, caught international attention. Due to its rapid spread throughout the Americas, authorities declared ZIKV a Public Health Emergency of International Concern that lasted from February to November 2016."Congenital Zika Syndrome" (CZS) is a new entity that reflects a broad spectrum of symptoms and signs observed in newborns infected by the ZIKV in utero. A recent review concluded that CZS differs from other congenital infections for its five distinct features: "(1) severe microcephaly with partially collapsed skull; (2) thin cerebral cortices with subcortical calcifications; (3) macular scarring and focal pigmentary retinal mottling; (4) congenital contractures; and (5) marked early hypertonia and symptoms of extrapyramidal involvement."Diagnosing and managing CZS has become a challenge for health professionals including ophthalmologists. The ocular manifestations of CZS are unique and have important consequences on vision. This article reviews the ocular manifestations of the CZS, addresses the visual outcomes of affected infants, and elucidates the early intervention protocols for visual improvement.