From 1990 to 2009, at the Research Institute of Children Oncology and Hematology have undergone examination and treatment 26 children with paratesticular rhabdomyosarcoma. The average age of our patients was 6.2 years (range 3 months to 15 years). All children was performed tumor markers level evaluation and ultrasound. In 6 children identified metastases. Surgical treatment is the first stage in the volume of orchifunikulektomia was held in 26 children. Retroperitoneal limfoadenektomiya was performed in 4 children and 1 child undergone thoracotomy with removal of metastases in the lung. Drug treatment was performed in 26 children with paratesticular rhabdomyosarcoma tumor. Using a combined method of treatment of malignant tumors led to 92.3 % overall survival.