Soft tissue sarcomas are a heterogeneous group of non-epithelial extraskeletal malignancies accounting for about 8 % of childhood cancers. Half of the cases are represented by rhabdomyosarcoma, a typical embryonal tumor of childhood, characterized by high grade of malignancy and strong propensity to metastasize, but also by a general good response to chemotherapy and radiotherapy. The group of “non-rhabdomyosarcoma soft tissue sarcomas” includes a variety of malignancies with different biology, grade of malignancy, and clinical course, most of them being tumors more common in adults, generally considered being relatively insensitive to chemotherapy. In most cases, the treatment of pediatric soft tissue sarcomas is complex and necessarily multidisciplinary. The prognosis depends on multiple factors, including age, primary tumor site and size, lymph node involvement, histology, surgical resection, and distant metastasis: overall, approximately 70 % can be currently cured with the modern multidisciplinary and risk-adapted treatment approaches.