background: alkaptonuria (aku) or ochronosis is a rare progressive degenerative arthropathy that results from deficiency of enzyme homogentisate 1,2 dioxygenase (hgd). the features include arthritis of the spine and in larger peripheral joints, with chondrocalcinosis. in this paper, we present a case of alkaptonuria in a 54 year old woman in tehran, iran. case presentation: a 54 year old woman ...