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The aim of this systematic review to assess the global prevalence of congenital gastrointestinal (GI) anomalies. We conducted a systematic review of global population-level birth prevalence of gastroschisis, omphalocele, intestinal atresia, esophageal atresia, congenital diaphragmatic hernia, Hirschsprung\u2019s disease, and anorectal malformation. We identified 103 studies from high and middle-income countries. Gastroschisis was the most reported condition. Studies reported geographic heterogeneity in gastroschisis, omphalocele, esophageal atresia, Hirschsprung\u2019s disease, congenital diaphragmatic hernia, and anorectal malformation prevalence. The prevalence of gastroschisis, omphalocele, and Hirschsprung\u2019s disease prevalence was reported to have increased over recent decades. Mean prevalence per 10\u2009000 live and total births ranged from 2.1 to 4.4 cases and 1.4 to 3.6 per 10\u2009000 live or total births, respectively. 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