Alkaptonuria is a rare autosomal-recessive metabolic disease caused by congenital homogentisic acid (HGA) oxidase enzyme deficiency which affects one in 100,000 to 250,000 individuals. Circulating HGA pass into various tissues through-out the body, mainly in cartilage and connective tissues, where its oxidation products polymerize and deposit as a melanin-like pigment. Currently, there is no specific treatment for alkaptonuria. A 63-year-old male patient presented to our clinic, complaining of chronic hip pain that had worsened over the previous 3 years. The patient also had darkly stained sclera and pinnae characteristic of ochronosis. A cementless right total hip replacement was performed. At the 5-year follow-up, the patient had returned to full activities, reported no hip pain, and was very satisfied with the outcome.