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Soft tissue sarcomas constitute approximately 8–10 % of all malignancies in children and adolescents and half of them are rhabdomyosarcoma. The second half makes up a heterogeneous group of malignancies of mesenchymal non-rhabdoid origin, which have been recently referred to as an individual group of non-rhabdoid soft tissue sarcomas (an adult type) in increasing frequency. The choice of a treatment option depends on a number of factors: its histological structure, location, stage of a tumor; the possible performance of radical surgery; and the grade of tumor differentiation. Surgery is the basic treatment; if it is impossible, chemoradiotherapy is performed at stage 1.