Background & Objectives: Congenital abnormalities are essential causes of morbidity and mortality in neonates. Congenital disorders are diseases which develop during egg fertilization or during embryo development. The prevalence of affected infants that are born alive is estimated about 3-5%. .This study has been performed on revealed malformations of the infants born in Sistan region. Material & Methods: This descriptive cross-sectional analysis was performed on all infants born alive and the congenital anomalies were detected from 1800 infants in three months. Sex, gestational age, type of abnormality, and neonate's status at discharge were recorded in a questionnaire. The data was analyzed by the spss 19 and chi-square test software. Results: The prevalence of abnormalities was 1/8%. There was a significant difference between maternal age and the incidence of congenital anomalies (P≤0.05). According to our findings, the prevalence of congenital malformation especially abnormalcy of the ear, eye and face was 29.5%. Conclusion: The prevalence of congenital anomalies in year 1391 was higher than 1383 in Sistan region. Thus, more medical attention should be paid to their diagnosis and treatment at early stage. Also educational programs on congenital malformations and the consequences of consanguineous marriages should be performed.

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