Alkaptonuria is a rare autosomal recessive metabolic disorder caused by the lack of homogentisic acid oxidase enzyme. As alkaptonuria can affect multiple systems, musculoskeletal manifestation leads to degenerative changes of the spine and peripheric joints. In this article a case with the complaint of low back pain who diagnosed as ochronotic spondylosis and arthropathy in the light of clinical, radiologic and laboratory findings is presented and discussed with the review of literature. ©2008, Firat University, Medical Faculty.