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A 72-year-old woman who had been diagnosed as homozygous familial hypercholesterolemia was admitted for chest discomfort. Computed tomography and cardiac catheterization revealed severe calcification of the aortic root and a high grade stenosis of the proximal right coronary artery. Aortic valve replacement concomitant with coronary artery bypass was done using temporary hypothermic circulatory arrest. This is preferred method when dealing the calcified aorta.