骨外尤文肉瘤 (EES) 是一种罕见的实体，仅占上肢病变的 3%。我们介绍了两名儿科患者，根据临床评估和影像学，他们最初被诊断为血管畸形。最终的组织病理学显示软组织起源的尤文肉瘤，经免疫组织化学分析证实。手外科医生经常因各种手部病变而就诊，因此在治疗此类病变时应保持警惕，并将 EES 视为一种鉴别诊断。多学科方法和适当的治疗算法有助于快速诊断，改善疾病的长期预后。证据级别：V 级（治疗）。

Extraskeletal Ewing sarcoma (EES) is a rare entity, accounting for only 3% of lesions encountered in upper extremity. We present two paediatric patients, who were initially diagnosed with a vascular malformation based on clinical assessment and imaging. Final histopathology revealed Ewing sarcoma of soft tissue origin, confirmed by immunohistochemical analysis. Hand surgeons, who are routinely approached for a myriad of hand pathologies, should be wary and consider EES as a differential when treating such lesions. A multidisciplinary approach with an appropriate treatment algorithm can help in a speedy diagnosis, improving the long-term prognosis of the disease. Level of Evidence: Level V (Therapeutic).