We report findings in a male infant born with talipes equinovarus and ischemic necrosis of the left lower leg and foot associated with atypical coarctation of the ascending aorta and stenosis and hypoplasia of the proximal innominate artery. We hypothesize that a vasculopathy of unknown etiology occurred in utero that resulted in ascending aortic arch coarctation and innominate artery stenosis. The presence of a thrombus in the coarcted segment suggests that one or more emboli could have been responsible for vascular compromise and subsequent clubfoot deformity and lower leg ischemia. Alternatively, a vasculopathy similar to that hypothesized as occurring in the aorta might also have occurred in the left anterior tibial artery, causing structural deformity and soft tissue ischemia of the left lower leg and foot. We believe that this is the first report of this association of findings.