Background: Congenital limb DEFECTs (CLDs) are the leading cause of disability across the world. CLDsvary depending on the anatomical location, type, and cause of anomaly. The present study aimed to evaluate the prevalence and patterns of CLDs in the North of Iran. Methods: This descriptive-analytical, hospital-based study was conducted in three hospitals in Gorgan, the capital of Golestan Province in the north of Iran. Samples included 32, 895 newborns with CLDs, and stillborn neonates were excluded from the study. Data analysis was performed in SPSS version 16 using Chi-square at the significance level of ≤ 0. 05. Results: Overall prevalence ofCLDs was three cases per 1, 000 live BIRTHs, and the rate was estimated at 3. 17 and 2. 82 cases per 1, 000 live BIRTHs in male and female infants, respectively. In addition, the prevalence of upper-and lower-limb anomalies was 1. 03 and 1. 91 cases per 1, 000 live BIRTHs, respectively. In terms ofethnicity, the prevalence of CLDs among the native Fars, Turkmen, and Sistani populations was 3. 86, 2. 02, and 3. 85 cases per 1, 000 live BIRTHs, respectively. Clubfoot was the most common type of CLDs, and the most prevalent type of the associated malformationswas gastrointestinal anomalies. Conclusion: According to the results, the prevalence of CLDs in the north of Iran was similar to European countries (2. 11-3. 18 cases per 1, 000 live BIRTHs), while it was lower compared to the other regions in Iran (5. 8 cases per 1, 000 live BIRTHs).