Background <p>The primary objective of this study is to define the global productivity of Hirschsprung disease (HSCR), identify influential studies, determine research directions focused on both historical and contemporary perspectives.</p> Methods <p>The study obtained 2816 articles published between 1980 and 2023 related to HSCR from the Web of Science database, and comprehensive bibliometric analysis were conducted.</p> Results <p>The top three most productive countries were the USA (<i>n</i> = 1283), China (<i>n</i> = 1167), and Japan (<i>n</i> = 587). The most productive institution was Université Paris Cité (<i>n</i> = 149), and the most productive author was Prem Puri (<i>n</i> = 99). The most frequently used keywords in the articles were enterocolitis (<i>n</i> = 191), enteric nervous system (<i>n</i> = 136), and transanal endorectal pull-through (<i>n</i> = 129).</p> Conclusion <p>Academic interest in HSCR began in 1887-1888, significantly increased in 1948 and 1964, and reached its highest peak in 1994. The findings indicate that key topics such as enterocolitis, enteric nervous system, surgical techniques, and genetic factors have been prominent in HSCR research. Previous studies, which often focused on molecular and genetic mechanisms, have shifted towards clinical and surgical applications in the last decade. Factor analysis reveals the complexity and diversity of HSCR research, with various topics examined, including clinical features, surgical treatments, pathological findings, and genetic bases.</p> Impact statement <p><UnorderedList Mark="Bullet"> <ItemContent> <p>This study presents a comprehensive bibliometric analysis of global research trends and future directions in Hirschsprung disease, revealing significant changes and developments in the study of the disease.</p> </ItemContent> <ItemContent> <p>The study provides an important contribution to the existing literature by detailing the historical development, main research topics, and thematic evolution of Hirschsprung disease research.</p> </ItemContent> <ItemContent> <p>The findings indicate that future research focus in Hirschsprung disease may increasingly prioritize innovative approaches, such as stem cell therapy, alongside clinical and surgical advancements.</p> </ItemContent> <ItemContent> <p>These advancements have the potential to enhance patient quality of life and guide future research strategies.</p> </ItemContent> </UnorderedList></p>