Background. Congenital anomalies are responsible for a remarkable proportion of mortality and morbidity in newborns. The aim of this study was to document the epidemiological features of congenital anomalies in rural areas, northwest of Iran. Method. The study population included live births born between 2004 and 2012 in rural areas of Tabriz district. All health records of the children under 8 years were assessed retrospectively. Results. Of 22500 live births, 254 cases were identified with a primary diagnosis of congenital anomalies giving a prevalence rate of 112.89 per 10 000 births (95% CI: 99.08 to 126.69). Anomalies of the nervous system were the most common defects, accounting for 24% of birth defects followed by the heart diseases anomalies. The highest prevalence rate for birth defects was observed in the south-western region with 386 per 10 000 births (95% CI: 215 to 556) compared to the similar rate in the north-western region with 15 per 10 000 births (95% CI: -14 to 45). Conclusion. The considerable geographic disparities in the prevalence of congenital anomalies in the region might be attributed to the highly polluted industrial zone in the area (including air and water pollution, etc.). This needs further etiological investigations in the region.