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Background and Objective: Birth defects are important causes of childhood morbidity and disability. This study was done to determine the incidence and pattern of birth defects in live birth in cities of Golestan province, north of Iran. Methods: This descriptive study was carried out on 92420 live births in 13 hospitals in Golestan province, northern Iran from 21 January 2008 to 20 March 2011. The newborns were examined for the presence of birth defects. Gender, type of birth defects and residency of parents according to city in Golestan province was recorded for each newborn. Results: The incidence rate of birth defects, in Gorgan, Kordkoy, Aliabad and Gonbad was 20.46, 12.53, 10.86, and 8.99 per 1,000 live births, respectively. The incidence rate of birth defects western area (including Kordkoy, Bandargaz and Kordkoy), center (Gorgan, Capital city) and eastern area (including Aliabad, Gonbad, Minodasht and Kalaleh) of Golestan province) was 9.3, 20.46 and 8.79 per 1,000 live births, respectively. Cardiovascular anomaly was the most frequent birth defects. Conclusion: The incidence rate of birth defects varies in diferent area of Golestan provine and overally was lower than the other region in Iran.