Nonatherosclerotic etiologies of arterial insufficiency are uncommon but important causes of chronic lower extremity ischemia. We report a patient with multiple hereditary exostoses (MHE) presenting with lifestyle-limiting lower extremity claudication and popliteal artery occlusion secondary to a large osteochondroma. The presence of MHE with associated osteochondroma resulting in arterial occlusion is a rare condition. Management strategies for treating large osteochondromas adjacent to or with vessel involvement in asymptomatic patients remain undefined.