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Objective: To study the prevalence and types of congenital anomalies that present at birth in a secondary-level hospital in South India and its contribution to perinatal mortality.Materials and methods: A total of 36,074 births over 10 years, from 2003 to 2013, were studied for the prevalence of gross congenital malformations at birth. It was a descriptive, cross-sectional study using data from the birth register and available medical records.Results: The incidence of birth defects was 12.5 per 1000 live births, with musculoskeletal disorders being the commonest, followed by craniovertebral anomalies. The prevalence of anomalies over the past 10 years has not shown any significant change (p= 0.555).Discussion: The high prevalence of neural tube defects indicates the need for periconceptional folic acid supplementation and early detection of anomalies, which would help in timely management. Detection of musculoskeletal anomalies would help in counseling patients antenatally.