Valvular and perivalvular involvement in end-stage renal disease (ESRD) is most commonly manifested as mitral annular calcification and aortic valve calcification. Both mitral and aortic valve calcification (MAC) occur more frequently and at younger age in those with ESRD than in those with normal renal function. Aortic valve calcification progresses to aortic stenosis more commonly and at a more accelerated rate than in the general population. Altered calcium and phosphate metabolism are thought to predispose to these valvular and perivalvular abnormalities. No treatment is necessary for MAC unless severe mitral regurgitation or stenosis occur (both are rare). Mitral valve repair or replacement and aortic valve replacement are indicated for severe symptomatic valve stenosis or regurgitation, albeit at a higher risk than in those with normal renal function. Infective endocarditis may complicate MAC or aortic stenosis in patients with ESRD and is associated with a high mortality rate in such patients.