In 2015, in Northeast Brazil, cases of microcephaly associated with the outbreak of zika virus in South America were identified. This virus is transmitted by the biological vector Aedes aegypti, which also transmits dengue, yellow fever, and chikungunya viruses. The congenital Zika syndrome affected newborns with head circumference smaller than 32 cm, compromising their central nervous system. Besides ocular lesions, hyperexcitability, and hypertonia, newborns also showed intracranial calcifications in imaging exams. If children with congenital Zika syndrome suffer maxillofacial alterations during embryonic development, they could have delayed tooth eruption and malformations. This study aims to perform an integrative review of the current literature about the health conditions of children with congenital Zika virus infection and the possible alterations of their oral cavity. This integrative literature review was performed by searching scientific articles in the PubMed, SciELO, and MEDLINE electronic databases with the descriptors “Zika Virus Infection”, “Zika Virus”, and “Microcephaly”, which deal with clinical case reports and were indexed between 2016 and 2021. About 26 articles published entirely in English were analyzed after meeting the eligibility criteria. The studies contributed to better treatment conducts by showing the neurological, functional, and oral alterations in children with congenital Zika syndrome, including periodontal diseases, malocclusions, dental caries, bruxism, micrognathia, ankyloglossia, short labial frenum, dental trauma, and dysphagia.