Introduction A pathological fracture in patients with primary bone sarcomas is a complication that requires specific management with the prognosis affecting the disease. The treatment strategy of the patients with pathological fracture remains controversial. The aim was to explore the effect of a pathological bone fracture on treatment of pediatric bone sarcoma. Material and methods The study included 141 children with bone sarcomas, residents of Moscow. A pathological fracture was observed in

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... 7 (12.1 %) cases accompanied by osteosarcoma in 10 (58.8 %) cases and Ewing's sarcoma in 7 (41.2 %) cases. Long bones were most common location of the fracture seen in 15 (88.2 %) cases. Results Errors in establishing the diagnosis of bone sarcomas occurred in children with/without a fracture with no statistically significant differences (p = 0.239). There were no statistically significant differences in the volume of surgical interventions, functional assessment using the Musculoskeletal Tumor Society System (MSTS), and 5-year survival in children with/without a fracture (p ≥ 0.05). Discussion Treatment of pathological fractures in children should be produced after obtaining biopsy and the histological diagnosis. The frequency of local recurrence and functional outcome does not depend on the fracture. Conclusion Pathological process is to be ruled out in the presence of a fracture, and adequate visualization using MRI to be followed by a biopsy in a specialized institution would be needed if a pathological focus is suspected.