Background: Kaposiform hemangioendothelioma (KHE) is an aggressive vascular tumor that primarily affects young children. This study aims to comprehensively analyze research trends and review current clinical treatments in KHE. Materials and methods: Publications related to KHE from 1993 to 2024 were retrieved from the Web of Science Core Collection database and analyzed bibliometrically using VOSviewer, CiteSpace, and the R package “bibliometrix.” Additionally, studies involving clinical treatment were further reviewed to analyze therapeutic efficacy and associated adverse events (AEs). Results: A total of 320 publications were included. An overall upward trajectory in annual publication counts was observed. The majority of top-cited papers originated from hospitals in the United States. Pediatric Dermatology was the most productive journal, and the most co-cited journal was the Journal of Pediatrics . The United States was the most collaborative, while China was the most productive. Sichuan University ranked as the top research institution, with Yi Ji from its affiliated West China Hospital having the highest H-index. Current research topics mainly focus on clinical characteristics and sirolimus treatment of KHE. Regarding the clinical treatments for KHE, sirolimus has emerged as the primary drug for KHE treatment due to its superior efficacy over other agents, with upper respiratory tract infections and oral mucositis being the most common AEs. Conclusion: This study is the first bibliometric analysis to provide a comprehensive overview of current research topics and trends in KHE. In addition, our results showed that sirolimus has emerged as the primary drug for KHE treatment.