Giant urethral stones in male are rare clinical entities and majority of them migrate from the upper tract. Rarely, urethral stones formed primarily in the urethra and are usually associated with urethral strictures or diverticula. We report a 56-year-old man with giant urethral stone associated with extremely rare urethral cancer. The etiologic factors, pathogenesis, clinical presentation, complications and management of giant urethral calculi are reviewed.