We report a case of urethral malacoplakia associated with disease of the bladder and bladder neck. Treatment consisted of fulguration of the bladder lesions and excision of the urethral lesion. Unlike most other cases of genitourinary malacoplakia, which are associated with chronic urinary tract infection, this patient had sterile urine and no history of urinary tract infections. Urethral malacoplakia is reviewed, and the pathogenesis and treatment of the disease are discussed.