Biliary atresia (BA) is a rare cholangiopathy of largely unknown etiology and unpredictable outcome. There has been an increasing number of BA-related publications, which may challenge researchers to determine their actual scientific value. This study aimed to evaluate the global research activity and developments relating to BA using a combination of scientometric methodologies and visualization tools. A comprehensive search strategy for the Web of Science TM database was designed to obtain bibliographic data on scientific BA publications for the timespan 1900-2018. Research output of countries, institutions, individual authors and collaborative networks was analyzed. Semi-qualitative research measures including citation rate and h-index were assessed. Choropleth mapping and network diagrams were used to visualize results. In total, 4,459 publications on BA were identified (88.5% in English), originating from 63 countries. The largest number was published by the USA (n = 991; 22.2%), Japan (n = 667; 15.0%) and the UK (n = 294; 6.6%). The USA combined the highest number of cooperation articles (n = 140). The most productive collaborative network was established between the USA and Canada (n = 17). Scientific papers from the UK received the highest average citation rate (16.7), whereas the USA had the highest country-specific h-index (59). Eighty-eight (2.0%) items were published under the auspices of multicenter consortiums and registries. The most productive institutions and authors were based in the USA, the UK, Japan, France, Canada and Taiwan. BArelated research has constantly been progressing, becoming more multidisciplinary but with main research endeavors concentrated in a few high-income countries. Studies into pathogenesis of BA remain uncommon, but are sorely needed to foster true scientific progress with this rare disease. Hence, international collaborative and translational research should be strengthened to allow further evolution in this field.