Abstract Zika virus was a little known virus until recent years, when its introduction to South America caused a widespread epidemic. The subsequent unprecedented number of cases with congenital microcephaly and neurological abnormalities in newborn babies prompted the declaration of a public health emergency of international concern. Since then, through concerted efforts by the international research community substantial knowledge has been gained about the epidemiology and physiopathology of this disease. However, the full spectrum of this condition and long term sequelae in the population of children with congenital Zika virus syndrome are as yet unknown. Here we summarize key facts known on Zika virus and congenital Zika syndrome to date and current guidance for the treating clinician.