RESUMO
Rhabdomyosarcoma is the most common tumor affecting the lower urinary tract of children, and is highly malignant with a tendency to involve local structure early and eventually metastasize to distant sites. Although about 15% of all juvenile rhabdomyosarcoma involve the urogenital tract most often the prostate, bladder, only a small number begin in the paratesticular tissues. Paratesticular rhabdomyosarcoma usually presents as a large intrascrotal mass that compresses the testis and epididymis, sometimes reaching the external inguinal ring. None case of paratesticular rhabdomyosarcoma has previously been reported in literature yet in Korea. We fist report a case of this tumor developed in a 6 years old boy and the relevant literature has been reviewed.