Observations upon a rarely met congenital disease is reported, developing symptomless up to the age of 40. The degenerative alterations of the big joints and the spine are of a special interest as well as the changes in urine, ear and nose cartilages, scleras and the skin in the armpits. A relation is supposed between the deposition of homogentisic acid and the functional changes in the vessels, latent diabetes and renal complaints.