Tehran University of Medical Sciences Journal of Orthopedic and Spine Trauma 2538-2330 6 4 2021 03 29 108 110 Mahlisha Kazemi Department of Orthopedic and Trauma Surgery, Shariati Hospital, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran Hamid Rabie Department of Orthopedic and Trauma Surgery, Shariati Hospital, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran Mohammad Hasan sharafi Department of Orthopedic and Trauma Surgery, Shariati Hospital, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran 2020 11 08 2021 03 03 Background: Ochronotic arthropathy is a rare complication in patients with alkaptonuria (AKU) that occurs as a result of ochronotic pigment accumulation in the joints.   Case Report: This case report presents a 54-year-old patient with severe degenerative arthritis who underwent total hip arthroplasty on both sides within an interval of three years. The diagnosis was made by observation of a dark capsule and femoral head during the second surgery. The surgical treatments significantly reduced the pain and improved the range of motion (ROM).   Conclusion: Orthopedic surgeons must be vigilant about clinical manifestations of this rare condition, before and during the surgery. Arthroplasty is a favourable therapeutic option in patients suffering from ochronotic arthropathy. https://jost.tums.ac.ir/index.php/jost/article/view/232 https://jost.tums.ac.ir/index.php/jost/article/download/232/205