ObjectiveTo understand research hotspots and future development trends in the field of familial exudative vitreoretinopathy (FEVR) from 2014 to 2023. MethodsRelevant literature on FEVR was retrieved using the Web of Science Core Collection (SSCI and SCI-Expanded) from the Institute for Scientific Information. The bibliometric analysis software CiteSpace 6.2.R3 was used to analyze countries or regions, institutions, authors, co-cited references, and keywords. ResultsA total of 316 FEVR-related articles were included. The annual number of publications in this field showed a fluctuating upward trend from 2014 to 2023, with the highest number of publications in 2022, 51 papers (16.14%, 51/316); and the lowest in 2015, 15 papers (4.75%, 15/316). China had the highest number of publications, with 137 papers (43.35%, 137/316). Among institutions, Shanghai Jiao Tong University ranked first with 43 papers, while Professor Zhao Peiquan from Xinhua Hospital of Shanghai Jiao Tong University, had the highest number of publications among authors, with 34 papers. The country with the highest betweenness centrality was the United States, 0.91; the institution was the Chinese Academy of Medical Sciences, 0.16; and the author was Ding Xiaoyan, 0.12. The 316 papers were clustered into four research areas: #0 clinical characteristics, #1 ndp, #2 norrie disease, and #3 retinopathy of prematurity. Keywords such as "Chinese patients," "TSPAN12," "variants," and "spectrum" remained highly frequent up to 2023. ConclusionsThe number of publications on FEVR research from 2014 to 2023 show a growth trend, with Chinese research institutions and scholars contributing the most. Research on pathogenic genotypes and clinical phenotypes remains a crucial direction for future development.