Background <p>Ewing sarcoma (ES) is a rare type of cancer that primarily affects children and young adults. In recent years, there have been notable advancements in the treatment of ES due to the introduction of neoadjuvant chemotherapy and targeted therapy, as well as a wealth of related studies in this field published by scholars around the world. However, there is still a lack of a comprehensive report to aid researchers in quickly understanding the knowledge structure and emerging trends of this area.</p> Materials and Methods <p>We conducted a comprehensive search in the Web of Science Core Collection for relevant studies published from 2000 to 2023. To conduct a thorough scientometric analysis and create network visualizations, we utilized three different tools, namely, an online analysis platform, CiteSpace, and VOSviewer. These tools enabled us to examine various aspects such as annual publication output; active journals; contributions from countries, institutions, and authors; references; and keywords.</p> Results <p>A total of 5163 qualified publications were included. The annual number of publications on ES showed a significant linear growth trend (<i>R</i>²&#xa0;=&#xa0;0.927). The USA stands out as the most productive country. The institution and author that contributed the largest number of papers were University of Texas System and Dirksen U., respectively. As can be seen from highly cited studies and reference analysis and application of Surveillance, Epidemiology, and End Results (SEER) database, preclinical and clinical trials of multiple combination treatment strategies, especially with different approach of immunotherapy such as CAR-T cells or anti-PD-1 antibody for ES, are the primary focus of the current study. Keyword analysis identified several research topics including immunotherapy, tumor microenvironment, microRNA, biomarkers, EWSR1, metastasis, epigenetics, epithelial mesenchymal transition, mesenchymal stem cell, and combination therapy, which have emerged as frontiers in the field and hold significant potential as ongoing research hotspots.</p> Conclusions <p>ES is a field that has continued to receive increasing attention in recent years. Harnessing current immunotherapy approaches to improve outcomes of patients with ES is an area of intense interest. This study has provided a comprehensive knowledge map, development landscape, and future directions for ES research, and offered a practical and valuable reference for scholars to obtain better understanding of the current state of research in this field.</p>