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The treatment of pathological femoral neck fracture in children due to osteopetrosis is not clear as only a few cases have been reported to date. We encountered a 7-year-old osteopetrotic girl with asynchronous bilateral femoral neck fractures. Radiographical assessment showed markedly dense bones with loss of corticomedullary differentiation, sandwich vertebrae and increased density of the ribs. Her mother had a similar radiological picture. Genetic testing showed CLCN-7 mutation which confirmed the diagnosis of autosomal dominant osteope