While death due to ZIKV infection has also been described, reports of fatal cases have been infrequent, and no systematic reviews have been published.We performed a systematic review of the literature in four databases to assess fatal outcomes of postnatal ZIKV infection and the available evidence that links ZIKV infection.311 articles were retrieved, 20 of them were epidemiological reports from surveillance agencies and Ministries of Health. After screening by abstract and title, 59 articles were selected for full-text assessment. Of the 59, 35 were excluded with reasons, and 24 were finally included for qualitative analysis. We found a total of 51 reported deaths associated with ZIKV infection in nine countries, the majority (56.9%) was not related to Guillain-Barré syndrome. Cases from three countries accounted for 67.6% of the deaths. ZIKV infection in the majority (64.7%) of cases was laboratory-confirmed.ZIKV was not considered to be a dangerous, much less a lethal pathogen until very recently. However, an increasing number of fatalities have been published in the literature since the first death was reported in 2016. Additional research is needed to elucidate factors that may mediate the pathogenesis of severe, atypical and fatal disease.