Osteopetrosis is a heterogeneous group of heritable conditions in which there is a defect in bone resorption by osteoclasts. It is associated with an increased skeletal mass due to abnormally dense, but brittle, bones. Osteopetrosis varies greatly in severity, and fracture treatment remains a matter of controversy due to altered responses to fixation and the risk of osteomyelitis. The fate of sternotomy in this condition is unknown, and osteopetrosis could represent a situation of 'hostile chest'. Here, the case is described of a patient with osteopetrosis and concomitant symptomatic aortic valve stenosis and coronary artery disease.

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