Purpose: To determine the clinical and functional outcomes of children undergoing limb-sparing therapy for extremity sarcomas, Methods and Materials: We retrospectively reviewed 30 patients, age less than or equal to 21 years, who were treated between 1979 and 1998 with external beam radiotherapy as a component of limb-spacing therapy for primary sarcomas of the extremity at UCSF. Included were patients for whom complete follow-up and functional outcome assessments were available. We assessed the patterns of failure, overall survival, disease-free survival, local control, and limb function. Results: At a median follow-up of 3 gears, 12 of the 30 patients recurred: 3 locally, 8 distantly, and 1 with synchronous local and distant disease as site of first progression, Eighteen patients were alive with no evidence of disease, The median overall survival was 10 years, with a median disease-free survival of 8 years. Functional outcome assessment revealed 15 patients retained excellent, 12 good, 1 fair, and 2 poor limb function. Conclusion: in pediatric patients receiving limb-sparing therapy, 90% maintained excellent or good limb function without compromising survival, demonstrating the validity of limb preservation in children with extremity sarcomas, (C) 2001 Elsevier Science Inc.