Endogenous ochronosis, a rare inherited disease of tyrosine metabolism, is
caused by a deficiency of the enzyme, homogentisic acid oxidase, and may le
ad to cardiovascular involvement seen most frequently as aortic valve steno
sis. We report the case of a patient with generalized ochronosis who develo
ped cardiovascular symptoms due to aortic valve stenosis and who underwent
aortic valve replacement.