[Alkaptonuria, prostatic calculi, and ectopic ureter]

Alkaptonuria is an uncommon tyrosine metabolism disorder. Deficit of homogentisic acid oxidase leads to the elimination of large amounts of homogentisic acid in the urine with accumulation of homogentisic acid oxidized pigment in the connective tissue (ochronosis). The ultimate evolution of ochronosis is degenerative arthritis. The clinical case reported is a 57-year old male diagnosed with alkaptonuria in an early stage of the connective tissue disease who comes to the clinic due to a lower urinary obstructive syndrome secondary to benign prostate hyperplasia but is diagnosed with giant prostate lithiasis. The patient undergoes retropubic adenomectomy with lithiasis removal and re-implantation of ectopic ureter from a dual left excretory system. Clinical evolution is completely successful. The rarity of the case calls for circulation and revision of the clinical and therapeutical aspects of this entity.