Introduction: Anencephaly is a common __ampersandsignamp; lethal neural tube defect (NTD) which occurs due to the defective closure of rostral pore of neural tube. The study aims to identify the risk factors associated with anencephaly in our population. Attempt was made to correlate the incidence of anencephaly with associated systemic and congenital anomalies, maternal factors, biochemical analysis of mother for ACE, alpha fetoprotein, folic acid __ampersandsignamp; random blood sugar. Material __ampersandsignamp; Method: The present study included 60 anencephalic fetuses of 20-30 weeks __ampersandsignamp; mothers of the fetuses from Victoria __ampersandsignamp; Vani Vilas hospital attached to Bangalore Medical College __ampersandsignamp; Research Institute. Study was conducted over a time period of 3 years from August 2014 to June 2017. Results: The incidence of anencephaly in Victoria and Vanivilas hospital was 1.04 in 1000 births. 23 (38.4%) were males __ampersandsignamp; 37(61.6%) were females. The alpha fetoprotein is high in 100% of cases __ampersandsignamp; ACE level was normal in 85 % cases, in 1.6% it was high __ampersandsignamp; in 13.4% cases it was low. In 15%, folic acid was low __ampersandsignamp; 15% cases were hyperglycemic. Conclusion: Better knowledge of unexpected fetal loss is the promise for better parental counseling __ampersandsignamp; for prevention of recurrences. Understanding __ampersandsignamp; identifying the risk factors associated with anencephaly in our population, allows approaches to avoid them __ampersandsignamp; there by lower the incidence of anencephaly in our population.