A 79-year-old woman was admitted to our hospital for ischemic necrosis of the right first toe. During having normal lipid profiles, such as low-density lipoprotein cholesterol and triglyceride, plasma levels of lipoprotein(a) (Lp(a) were abnormally high (141 mg/dL). She had a history of heart failure (HF) due to aortic valve stenosis (AS) and drug-eluting coronary stenting due to angina pectoris. To avoid worsening of HF and limb ischemia during minor amputation, she underwent balloon aortic valvuloplasty and endovascular therapy. She was also placed on proprotein convertase subtilisin/kexin type 9 inhibitors (140 mg of evolocumab) every 2 weeks, which decreased her plasma Lp(a) levels to 105 mg/dL (26% decrease) at discharge. Elevated plasma Lp(a) levels could strongly affect the development of AS and progression of systemic atherosclerosis. The screening and treatment of increased plasma Lp(a) are imperative for patients with AS having peripheral arterial disease.