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Mkpe Abbey,1 Olufemi A Oloyede,2 Goddy Bassey,1 Benjamin M Kejeh,3 Barbara E Otaigbe,4 Peace I Opara,4 Austa U Eneh,4 Chris I Akani1 1Department of Obstetrics and Gynaecology, University of Port Harcourt Teaching Hospital, Port Harcourt, 2Department of Obstetrics and Gynaecology, Olabisi Onabanjo University Teaching Hospital, Sagamu, 3Department of Surgery, 4Department of Paediatrics, University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria Objective: To ascertain the prevalence and pattern of congenital abnormalities that are peculiar to the Niger Delta area of Nigeria. Methods: This is a descriptive retrospective cross-sectional study. It involved data from the labor ward and neonatal birth registers of the University of Port Harcourt Teaching Hospital on the total number of births and the babies that were delivered with major birth defects between August 2011 and December 2014. We also conducted a statistical comparison of the prevalence of congenital abnormalities in the Niger Delta with that in other regions of Nigeria and the developed world of Europe. Results: Out of the 7,670 deliveries that occurred, 159 maternities had babies with major birth defects giving a prevalence of 20.73 cases per 1,000 live births. This figure is far more than that which was obtained in other regions of Nigeria –4.15:cases per 1,000 live births in the South East (P |