This integrative review aimed to analyze the incidence of congenital anomalies in neonates and identify associated risk factors, as well as explore the most effective preventive measures described in the scientific literature. Congenital anomalies are structural or functional defects that develop during intrauterine life and are a major cause of neonatal and infant mortality. An estimated 303,000 newborns die annually within the first month of life due to these conditions (WHO, 2016). This study was conducted through the analysis of scientific articles published in the PubMed, SciELO, and Google Scholar databases, using keywords such as "congenital anomalies," "neonates," and "risk factors." Studies published in the last ten years, in Portuguese and English, addressing the incidence and risk factors of congenital anomalies in neonates were included. The results showed that the most common congenital anomalies include neural tube defects, cardiac malformations, and chromosomal abnormalities, such as Down syndrome (PACHECO et al., 2009). The prevalence of these conditions varies according to geographic region and population (BRASIL, 2021). Several maternal risk factors were identified, including advanced maternal age, smoking, alcohol consumption, and exposure to teratogenic substances during pregnancy (ANDRADE et al., 2017). Additionally, adverse socioeconomic conditions and lack of access to quality prenatal care were highlighted as important determinants for the increased incidence of congenital anomalies (LANSKY et al., 2014). Preventive strategies suggested by the literature include folic acid supplementation before and during pregnancy, proper vaccination of pregnant women, and genetic counseling (BRASIL, 2013; ACGO, 2018). Early diagnosis through neonatal screening tests, such as the heel prick test, pulse oximetry, and hearing screening, is crucial for early identification of anomalies and enabling interventions that can improve health outcomes for neonates (MINISTÉRIO DA SAÚDE, 2021). The review also emphasized the importance of early interventions, such as corrective surgeries and supportive therapies, to improve the prognosis of neonates affected by congenital anomalies (CHRISTIANSON, 2006). These interventions can significantly reduce morbidity and mortality rates associated with these conditions, improving the quality of life of affected individuals. In conclusion, this integrative review identified significant risk factors for congenital anomalies and highlighted the importance of preventive interventions and early diagnosis to improve neonatal health outcomes. The effective prevention and management of congenital anomalies require a multidisciplinary approach, including awareness among healthcare professionals, effective public policies, and family involvement in implementing preventive strategies. Ongoing research is recommended to further understand the causes, risk factors, and effective interventions to reduce the incidence of congenital anomalies and improve neonatal health outcomes.