This report outlines the management of a large intrathoracic lesion found in a 2-year-old girl with hereditary multiple exostosis. The lesion arose from the right eighth rib and comprised two separate osteochondromata that had coalesced into a single lesion and caused significant deformity to the chest wall. Aside from the deformity, the lesions were asymptomatic. Further growth of the lesions could cause respiratory complications, worsening of the visible deformity and, being lesions of the axial skeleton, bear an increased risk of malignant change. The lesions and the attached eighth rib were removed operatively and the patient recovered without complications. We demonstrate a place for the operative management of asymptomatic lesions in anticipation of future difficulties or malignant changes.
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