Objective <p>Malformations of the craniovertebral junction (CVJ) have recently arisen as a focal point for research. Chiari malformation (CM) and basilar invagination (BI) are the most common CVJ malformations in adults. Conducting qualitative and quantitative analyses of the relevant literature are essential to understand these conditions. Therefore, the present study aimed to identify and summarize published articles related to CM and BI, and to analyze and visualize current research topics and trends.</p> Methods <p>Relevant studies related to CM and BI were extracted from the Web of Science Core Collection and PubMed database. Data were downloaded in plain text, Tab. Delimited File, and BibTeX formats. R software, Excel, VOSviewer, Scimago Graphica and CiteSpace were used to analyze data on countries, institutions, authors, references, and keywords to identify research hot topics and trends.</p> Results <p>In total, 1939 publications were included, while the number of related publications showed an annual increasing trend. The United States of America produced the highest number of publications globally. Capital Medical University and Goel Atul were the institution and author with the most publications, respectively. <i>World Neurosurgery</i> had the highest number of published studies. Keyword analysis identified “Posterior Reduction,” “System,” “Release” and “Outcomes” as current hot topics with potential as future research trends.</p> Conclusion <p>This study provides valuable insights into research topics and future development trends in CM and BI. Future research will require more frequent in-depth collaborations among countries, institutions, and authors to explore the pathogenesis and treatment of CM and BI.</p>