Background <p>Total colonic aganglionosis (TCA) is a rare but severe congenital disorder that impacts the colon’s ability to function properly due to the absence of ganglion cells. Investigating research trends in the diagnosis and treatment of TCA can offer valuable insights into advancements in clinical practices, surgical techniques, and future directions in pediatric gastroenterology.</p> Methods <p>Literatures related TCA were explored through a search in the Web of Science Core Collection (WoSCC) database, spanning the period from 1978 to 2024. Bibliometric analysis and data visualization were conducted using VOSviewer, CiteSpace, and the R package “bibliometrix.”</p> Results <p>A total of 281 articles were analyzed, accumulating over 4,010 citations. The USA led in productivity, with Paris City University as the top institution, and <i>Journal of Pediatric Surgery</i> emerged as the leading journal. Prem, Puri was identified as the most influential author. Current hotspots included genetic mutations, management, clinical guidelines, diagnosis, and pull-through for TCA. Keywords burst analysis showed emerging interests in “diagnosis” (2020–2024), “management” (2022–2024), and “pull-through” (2022–2024).</p> Conclusion <p>This study presents a bibliometric analysis of TCA, focusing on emerging research trends, influential publications, and global collaborations. The findings identify key advancements in the understanding and treatment of TCA, providing valuable insights for both clinical and basic research in pediatric gastroenterology.</p> Clinical trial number <p>Not applicable.</p>