The goal of this study was to determine outcomes related to limb salvage vs. amputation for treating high-grade and localized osteosarcoma in patients with pathological fractures. Literature search was conducted using Medline, Embase and the Cochrane Database. Two reviewers independently assessed all eligible publications. The primary outcome measurement was pooled odds ratio (OR) and 95% confidence interval (CI) for the risk of local recurrence, 5-year overall survival rate and metastatic occurrence calculated through the fixed-effects method. Seven eligible studies were identified, which included a total of 284 patients. The risk for local recurrence and 5-year overall survival rate did not differ significantly (P>0.05) between the limb salvage group and amputation group, with an OR of 1.48 (95% CI, 0.67–3.30) and 1.85 (95% CI, 0.86–3.98), respectively. The risk for metastatic occurrence differed significantly (P<0.05), with an OR of 0.30 (95% CI, 0.10–0.91). The occurrence of a pathological fracture is not regarded as an absolute contraindication to limb salvage in patients with high-grade and localized osteosarcoma. Limb salvage as an alternative for treating high-grade and localized osteosarcoma in patients with pathological fracture does not greatly increase the risk for local recurrence or 5-year overall survival rate compared to amputation and has a lower risk for metastatic occurrence.