Cardiac ochronosis is a rare complication of alkaptonuria, a disorder of 
tyrosine metabolism characterized by a triad of dark urine, pigmentation of 
tissues, and ochronotic arthropathies. When present, cardiac ochronosis 
generally affects the aortic valve, resulting in aortic stenosis. More rarely, 
it may affect the mitral valve and the coronary arteries. This report describes 
the case of a 67-year-old woman with a history of alkaptonuria with severe 
ochronosis of the coronary arteries and mitral valve who required coronary 
artery bypass and mitral valve replacement.