Large osteochondroma arising from chest wall and sternum is uncommon and presentation with airway compression is further uncommon. Here we present a case of large chest wall osteochondroma as a part of Hereditary multiple exostoses in a 9 years old boy presented with a history of stridor and shortness of breath. The bony mass of the right chest wall was extending up to a suprasternal notch and compressing the trachea. The case was successfully managed by initial femoro-femoral cardiopulmonary bypass under local anesthesia prior to the induction of anesthesia to prevent respiratory collapse, followed by debulking surgery was done.