The purpose of the study was to establish an algorithm for the treatment of pathological fractures in children. Pathological fractures can compromise radiographic and histological diagnosis. The need for histological diagnosis and indications for surgical treatment are not clearly defined. We reviewed our Centre’s Tumour Registry records of children who presented over the past 7 years with a fracture as the first manifestation of primary bone pathology. There were 23 patients (average age 12 years and 2 months). There were 9 fractures through simple bone cysts, all treated conservatively initially. All patients were subsequently treated with needle biopsy and bone marrow injection. Three patients suffered refracture and underwent flexible intramedullary nail fixation. There were 5 cases of fibrous dysplasia. Histological diagnosis was obtained in all cases, followed by prophylactic intramedullary nailing in 3 patients. There were 2 patients with giant cell tumour, 3 with aneurysmal bone cyst and one with chondroblastoma. Histological diagnosis preceded curettage and grafting in all cases. Finally, there were 3 patients with Ewing’s sarcoma of the femur. One underwent palliative intramedullary nailing for extensive local disease. The second patient was treated conservatively initially. She subsequently underwent segmental resection and vascularised fibular graft. The third patient underwent internal fixation in another unit for what was considered to be a benign lesion. The histological diagnosis of Ewing’s sarcoma was based on intra-operative specimens. Definitive surgery required wide resection and prosthetic replacement. We recommend that primary fixation of pathological fractures should be avoided until histological diagnosis is obtained. All lesions should be appropriately imaged and biopsied if aggressive characteristics are present. However, if radiographic appearances are reassuringly benign, biopsy can be delayed until conservative fracture management is completed. Definitive treatment of benign lesions with protective intramedullary nailing or curettage and grafting can follow frozen section under the same anaesthetic.