Objectives: CRISPR-Cas9, a revolutionary gene-editing technology, has had profound impacts across various biological fields. Its application in nephrology is gaining increasing attention. Despite the rapid development of research in this field, a comprehensive overview of the knowledge structure and research hotspots of CRISPR-Cas9 in nephrology has not yet been conducted. Methods: We conducted a bibliometric analysis of publications related to nephrology and CRISPR-Cas9 from 2014 to 2023 using VOSviewers, CiteSpace, and the R package “bibliometrix”. Results: Our analysis included 325 articles from 47 countries, primarily led by the United States and China. The number of publications related to nephrology in CRISPR-Cas9 is increasing year by year. Key research institutions include Harvard University, Udice French Research Universities, University of California, and UNIVERSITE PARIS CITE. These institutions have made significant contributions to the field, with numerous publications and citations. Conclusions: CRISPR-Cas9 has revolutionized nephrology by providing a precise and targeted approach to modify DNA sequences. This technology holds immense potential in identifying new drug targets for kidney diseases through unbiased screens and in vivo gene editing. The ability to manipulate genetic sequences with nucleotide-level precision opens up new avenues for understanding and potentially treating autosomal dominant polycystic kidney disease (ADPKD). Furthermore, the successful suppression of the A4GALT gene using CRISPR/Cas9 in a kidney organoid model offers hope for developing therapeutic strategies for Fabry disease nephropathy (FDN). The advancements made with CRISPR technology in nephrology have paved the way for further research and exploration of novel treatments and interventions for kidney diseases. This study provides a comprehensive analysis of the knowledge mapping of CRISPR-Cas9 in nephrology, hoping to provide valuable references for future research. This comprehensive analysis will serve as a valuable reference for future research in this rapidly evolving field.