Aim: The objective of this study is to define the incidence of chromosomal and congenital anomalies in neonates with exomphalos major and minor. Background: Incidence of major congenital anomalies varies from 35% to 81% in exomphalos. It is unclear whether these malformations are more common with exomphalos major. Material and Methods: The case notes of 82 antenatal diagnoses of exomphalos,made between January 1998 and December 2004,were retrospectively reviewed. Exomphalos major was defined as a defect 5 cm or greater and exomphalos minor a defect less than 5 cm in diameter. Results: There were 72 live births,6 still births,and 4 terminations of pregnancy. There was no statistical significance between exomphalos major and minor regarding mode of delivery,gestational age at birth,birth weight,major cardiac anomalies (21% vs 23% ),and renal and external genitalia abnormalities (11% vs 18% ). Chromosomal anomalies,syndromes,and dysmorphism were common in exomphalos minor 17 (39% ,P = 0.0001). Congenital malformations of the gastrointestinal tract (14% vs 27% ),central nervous system (0 vs 21% ),and Wilms’ tumor (0 vs 5% ) occurred commonly in exomphalos minor. Limb abnormalities (25% vs 5% ),ectopia cordis (11% vs 0),and bladder exstrophy (7% vs 0) occurred predominantly in exomphalos major. Mean follow-up was 34 months. Three neonates with exomphalos major died. Overall mortality was 4% . Conclusions: Chromosomal anomalies and syndromes occur more commonly in exomphalos minor. Exomphalos minor and major seem to have a predilection for associated anomalies of specific organ systems. This predisposition may help in counseling parents,planning investigations,and organization of multidisciplinary management strategy. Aim: The objective of this study is to define the incidence of chromosomal and congenital anomalies in neonates with exomphalos major and minor. Background: Incidence of major congenital anomalies varies from 35% to 81% in exomphalos. It is unclear whether these malformations are more Common with exomphalos major. Material and Methods: The case notes of 82 antenatal diagnoses of exomphalos, made between January 1998 and December 2004, were retrospectively reviewed. Exomphalos major was defined as a defect 5 cm or greater and exomphalos minor a defect less than 5 cm in diameter. Results: There were 72 live births, 6 still births, and 4 terminations of pregnancy. There was no statistical significance between exomphalos major and minor regarding mode of delivery, gestational age at birth, birth weight, major cardiac anomalies (21 % vs 23%), and renal and external genitalia abnormalities (11% vs 18%). Chromosomal anomalies, syndromes, and dysmorphism were common in exomphalos minor 17 (39%, P = 0.0 001). Congenital malformations of the gastrointestinal tract (14% vs 27%), central nervous system (0 vs 21%), and Wilms’ tumor (0 vs 5%) occurred commonly in exomphalos minor. Mean follow-up was 34 months. Three neonates with exomphalos major died. Overall mortality was 4%. Conclusions: Chromosomal anomalies and syndromes occur more commonly in exomphalos minor. Exomphalos minor and major seem to have a predilection for associated anomalies of specific organ systems. This predisposition may help in counseling parents, planning investigations, and organization of multidisciplinary management strategy.