Alkaptonuria is a rare autosomal recessive disorder of metabolism caused by 
deficiency of homogentisic acid oxidase and resulting in accumulation of 
homogentisic acid in collagenous structures. This causes the classic clinical 
triad: (1) homogentisic aciduria (urine blackens on standing when oxidized or 
alkalinized); (2) eumelanin-like pigmentation of skin, sclera, cartilages, etc 
and (3) degenerative ochronic arthropathies usually in the fourth decade of 
life. Other important but more rare consequences of alkaptonuric ochronosis are 
cardiovascular and urinary tract involvement. We present a case of ochronosis 
with multiple visceral involvement: skin (fingers, ear sclera), severe 
spondylarthropaty with extensive calcifications of intervertebral discs and 
reduced mobility, osteoarthritis of both knees, right hip ostonecrosis, 
cardiovascular involvement (severe stenosis and insufficiency of aortic valve 
that) and urinary tract involvement (nephrolitiasis)