Calcifying obliterative atherosclerosis isolated within the descending thoracic aorta causing subtotal vascular occlusion was associated with symptoms such as in aortic coarctation in a 56-year-old patient. Remarkable in this unique case is the atypical and isolated manifestation of atherosclerotic disease within the thoracic aorta, as well as the tumorous extent of luminal calcification. Differential diagnostic considerations had to include calcifying tumor of the aorta, remnants after aortitis or secondary calcified aortic dissection.