Alkaptonuria, also called endogenous ochronosis, is a rare metabolic autosomal
recessive disorder. It occurs by complete inhibition of homogentisic acid oxidase
enzyme having its deposition in various tissues. Male patient, 52 years old, sought
medical help complaining about progressive appearance of hyperchromic papules on the
lateral edge of the second finger of both hands for 02 years. He also complained
about darkening of urine, sperm and underwear. Incisional biopsy of second hand
finger and test for homogentisic acid in the urine results were positive. The
findings are compatible with the diagnosis of alkaptonuria. Given these findings,
treatment was initiated, followed-up by other specialties and he was advised to avoid
certain foods.