Alkaptonuria is a rare inherited genetic disorder of tyrosine metabolism 
characterized by a triad of homogentisic aciduria, ochronosis, and arthritis. 
The most common clinical manifestations of ochronosis involve the 
musculoskeletal, respiratory, airway, cardiovascular, genitourinary, cutaneous, 
and ocular systems. We report the perioperative anesthetic management of a 
56-year-old alkaptonuric patient, with multiple comorbidities scheduled, for 
revision total hip replacement. A review of her medical history revealed 
alkaptonuria, hypothyroidism, rheumatoid arthritis, hypertension, diabetes 
mellitus, and Pott's spine with disc prolapse. We want to highlight the need of 
thorough preoperative evaluation in patients of alkaptonuria, as it is 
associated with multiple comorbidities. The systemic involvement should 
determine the anesthetic plan. Caution should be exercised during positioning to 
prevent injury to the joints and the spine.