Background Despite neoadjuvant chemotherapy and wide surgical ablation, 15% to 25% of patients with primary osteosarcoma will relapse (local recurrence or metastases). Neither chemotherapy nor radiation therapy alone will render a patient disease-free without concomitant surgical ablation of relapse. We prefer excision of relapse when possible. However, it is unclear whether excision enhances survival. Questions/purposes We therefore determined (1) onset, location, and treatments for relapse; (2) postrelapse diseasefree survival of patients who underwent surgical ablation and those who did not; and (3) relapse-free interval between initial diagnosis and first relapse in survivors and in those who died of their disease.Methods We retrospectively reviewed 15 children who initially presented with localized, nonmetastatic extremity osteosarcoma and attained initial complete remission after neoadjuvant chemotherapy, wide local resection, postoperative chemotherapy, and subsequently developed disease relapse. Relapse occurred at a median of 28 months, although late relapse after 5 years occurred in three. We resected the recurrent tumor in nine patients and treated six nonoperatively.