Background <p>Pacific Island Countries and Territories experience disproportionately high rates of stillbirth, neonatal, and childhood deaths, yet the drivers of these outcomes remain unclear. The impact of congenital abnormalities within the Pacific has not been clearly established, despite these abnormalities being a leading global cause of infant and child mortality. This scoping review maps available literature to determine the current knowledge of congenital abnormalities in Pacific Island Countries and Territories.</p> Methods <p>Two independent reviewers searched six scholarly databases (PubMed, Scopus, CINHL, Proquest, Web of Science and Google Scholar) in addition to performing targeted web searches for grey literature. Searches were limited to sixteen Pacific Island Countries and Territories and extracted data was analysed as thematic summaries.</p> Results <p>Seventy-two sources were included in the final review. Papua New Guinea was the most represented Pacific nation in identified literature. Congenital abnormalities were commonly reported without description or specification to identify the type of abnormality, but congenital heart defects (<i>n</i> = 17) and neural tube defects (<i>n</i> = 13) were most often reported. The burden of congenital abnormalities between Pacific Island Countries and Territories was not comparable due to limited and varied data collection and reporting methods in the literature.</p> Conclusions <p>Our exploration of the body of knowledge on congenital abnormalities in Pacific Island Countries and Territories shows that there is an unequal representation of Pacific nations in literature focused on congenital abnormality research and reporting. This gap may reflect variations in the capacity for health research across different Pacific nations. The paucity of literature and generalised reporting of congenital abnormalities further demonstrates ongoing challenges to health surveillance pertaining to common causes of stillbirth, neonatal and childhood deaths in the region.</p>