A 64-year-old man with a history of intermittent claudication presented with chest pain. On admission, he had yellowish papules with a "plucked chicken" appearance on his neck. Histological examination of the biopsy specimen of the lesion revealed degeneration and calcification of elastic fibers in the dermis. His daughter also had similar histological changes in the skin. The diagnosis was pseudoxanthoma elasticum. Fundoscopic examination revealed angioid streaks. Electrocardiography showed inverted T waves in V2-V6, and echocardiography revealed hypokinesis in the anterior wall of the left ventricle. Emergency catheterization showed 90% stenosis in the left anterior descending coronary artery, total occlusion of the abdominal aorta, and severe stenosis in the right subclavian artery. He was treated medically and had no recurrent episodes of chest pain. A careful examination for cardiovascular diseases should be considered in patients with pseudoxanthoma elasticum.