Background: Congenital heart defects (CHDs) have a major role in neonatal mortality. Given the effect of CHDs on the infant mortality rate (IMR), knowing its prevalence is necessary for health planning The present meta-analysis aimed to investigate the prevalence of CHDs in neonates in Iran. Methods: This is a meta-analysis in which all articles published by Iranian researchers in Persian and English from 1986 to 2015 which contained the keywords of congenital anomalies, congenital heart defects, prevalence, newborn, and Iran were enrolled The articles quality was reviewed using the JBI checklist and the I2 heterogeneity index, and the articles were analyzed by Comprehensive Meta-Analysis, version 3. Results: Out of 1251 papers in the initial studies, 14 papers published from 1986 to 2015 were enrolled in the study according to inclusion and exclusion criteria. The overall prevalence of CHDs was 3.7/1000, which was significantly lower than its prevalence before 2008. Conclusion: Given the high prevalence of CHDs in Iran, further studies can address them better and more accurately aiming to prevent their occurrence.