Rhabdomyosarcoma is the most common soft tissue sarcoma and it accounts for 6% of all childhood malignancies. It derives from primitive mesenchymal cells which obtain the ability of diferentiation into skeletal muscle cells. In children younger than 10 years of age the most common primary sites are the head and neck area as well as the genitourinary tract while in older children and adolescents rhabdomyosarcoma can more often be found in the trunk area as well as on the extremities. In most cases, rhabdomyosarcoma presents as a painless mass whose specific clinical manifestations vary with the site of origin of the primary lesion. The diagnostic evaluation of rhabdomyosarcoma consists of laboratory tests (complete blood count, comprehensive metabolic panel including liver functions, urinalysis) and radiologic tumor imaging for determining the primary site as well as the initial extent of disease. Important prognostic factors in rhabdomyosarcoma treatment include the patient’s age, stage of the tumor, histologic subtypes and certain genetic characteristics of the tumor. Treatment of rhabdomyosarcoma should be assessed to each patient individually and it combines surgery, radiation and chemotherapy. With those forms of treatment it is possible to establish both local and systemic control of disease. Overall, RMS is curable in the majority of children receiving optimal therapy and it has a survival rate of over 70%.