Epithelioid sarcoma is an uncommon soft tissue tumor, most prevalent in the extremities of young adults. Youth has been described as a favorable prognostic factor. We describe four patients (two children aged 10 years and two adolescents aged 18 years) with extremity epithelioid sarcoma. Both 10-year-old children had rapid disease evolution and died 3 and 6 months after the diagnosis was made. Both tumors were strict diploid at DNA flow cytometry, but one had an abnormal karyotype (trisomy 2). Both adolescents are disease-free 18 months and 18 years after combined treatment consisting of local resection, regional lymph node dissection, and isolated regional perfusion with cytostatic agents.