Zika virus (ZIKV), first discovered in 1947, is the most recent member of the TORCH family. It usually causes an asymptomatic or mildly symptomatic disease in infected adults but can lead to severe brain abnormalities in fetuses who are infected in utero by vertical transmission of the virus through the placenta. The constellation of these fetal/neonatal abnormalities is named as congenital Zika syndrome (CZS). Although horizontal transmission of ZIKV is largely dependent on the presence of the vector (i.e. Aedes mosquitoes), women residing in non-endemic areas are still at risk of acquiring the infection once they travel to an endemic region or have unprotected sexual contact with an infected male. Therefore, it is important for physicians practicing in non-endemic regions to be familiar with the clinical and neuroimaging manifestations of CZS and to consider this diagnosis as a potential etiology for congenital microcephaly and other fetal central nervous system abnormalities.