AbstractThe European pediatric Soft tissue sarcoma Study Group analyzed all children with epithelioid hemangioendothelioma prospectively registered in the NRSTS‐05 (EUDRACT 2005‐001139‐31) and in MTS‐2008 (NCT00379457) studies: 10 patients with localized and one with metastatic disease. Median age was 14.3 years (range, 9.0–18.8). Local therapy was initial primary surgery in seven cases, and five patients received systemic therapy. No patients received radiotherapy. After a median follow‐up of 50 months (range, 6–176) for living patients, nine patients remain alive off therapy and two died. Five‐year progression free and overall survivals are, respectively, 77.1% (95% confidence interval [CI]: 34.5–93.9) and 74.1% (95% CI: 28.1–93.0).