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Fibro-osseous dysplasia is a developmental non familial, benign anomaly of bone development occurring in single or multiple bones, characterised by the replacement of normal bone by fibro-osseous tissue. We describe the case of a fourteen year old boy, which illustrates many of the difficulties confronting surgeons dealing with this uncommon condition. Complete surgical excision was required, before a definitive histological diagnosis could be established. We report the clinical, radiological and pathological features of this unusual case. We review the pathology of the condition and discuss the approaches to surgical management.