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Background : Anencephaly is a lethal neural tube defect which is due to the defective closure of rostral pore of neural tube. In more than 50% of cases it is associated with other systemic anomalies. Hence this study was undertaken to assess pathological parameters associated with anencephaly in particular attention to associated systemic anomalies. Materials and Methods: It is a study on 41 anencephaly fetuses conducted in the Department of Pathology. The period of study is from January 2001 to December 2011. Results: Out of 41 cases, 30 (73%) cases showed presence of systemic anomalies, 48.5% of the cases were observed in primigravida. Most common associated anomaly was spina bifida followed by gastrointestinal anomalies. Conclusion: Pathological examination of the abortus is essential to document the associated anomalies.