Zika virus (ZIKV), belonging to the Flavivirus family, has been documented to primarily circulate within the Latin American and Caribbean countries, with small clusters reported in Asia recently. It is transmitted to humans primarily by arthropods, but can also spread via sexual intercourse, blood transfusions, and from an infected mother to a child. This vertical transmission of the virus can give rise to a severe clinical entity, called congenital Zika syndrome, which encompasses mild to severe birth defects. As the number of reported cases of congenital anomalies associated with the Zika virus progressively increased, the emphasis of research into this syndrome became important within the scientific community. Since the declaration of the public health emergency in 2015, several studies have documented the sequelae of antenatal exposure to the Zika virus. While this is similar to other arthropod-borne viruses, a unique feature of ZIKV is the positive tropism towards the central nervous system. Congenital Zika syndrome is characterized by a spectrum of anomalies that can affect the hearing, vision, neurological, and thus, the development of a child. This article aims to highlight the unique nature of this arthropod-borne virus, with emphasis on the etiology, transmission, clinical features, treatment, and prevention of congenital Zika syndrome.