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Osteopetrosis (OP) is a rare heterogeneous group of inherited skeletal dysplasias characterised by osteoclast dysfunction, impaired bone resorption and poor bone remodelling. Three groups can be categorised on the basis of clinical findings. These include neurological symptoms, haematological abnormalities and renal tubular acidosis in the first group. Increased bone density, osteomyelitis and frequent fractures are the clinical findings in the second group, and the third group have normal life expectancy but may develop cranial nerve compression and osteomyelitis. Fractures in patients with OP are common and require appropriate pre-, peri- and postoperative management. The long bones are most frequently affected, fractures of the femoral neck and proximal (upper third) shaft being particularly common. This case report proposes possible operative fracture treatment in a patient with OP and highlights the potential perioperative pitfalls in this rare surgical population.