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İstanbul Ü. Onk. Enst. Pediatrik Onkoloji BD 27 cases, 18 males and 10 females, of biopsy proven RMS under 18 years of age were seen in the University of Istanbul, Oncology Institute, Pediatric Oncology and Radiation Oncology Departments during September 1989 and December 1992. The median age was 5 years (7 mo.-18 yrs). The primary tumor was localized in the orbital region in 3 cases (11%), in the nonparamenengeal head and neck region in 1 (4%), in the paramenengeal head and neck region in 5 (18%), in the genitourinary tract in 8 (30%), in the extemities in 4 (15%) and in other regions in 4 (15%) cases. In two metastatic cases, the primary tumor localization could not be stated. Histopathologically, 15 cases (56%) were subclassified as embryonal, 5 (19%) as botryoid, 5 (19%) as alveolar; 2 cases could not be subclassified. Among 23 cases staged according to the IRS Clinical Grouping System, 3 (%13) in group I, 3(%13)in group II, 15 (65%) in group III, 2 (9%) in group IV. Eighteen percent of the cases had additional congenital malformations; 33% had a history of cancer in their family. Patients were treated by a protocol in which a combination of surgery, chemotherapy and radiotherapy were utilized. Fourteen cases (52%) are alive, seven (26%) have died, and 6 (22%) are lost to follow-up. The survival rate was calculated as 73.8% at 31 months of follow-up. Keywords :