Autosomaldominant polycystic kidney disease (ADPKD) is one of the commonest hereditary disorders and the most common life-threatening genetic disease, affecting an estimated 12.5 million people worldwide. PKD are congenital and bilateral diseases, and those symptoms almost never appear until after age 40. Urinary tract infection occurs with increased frequency in ADPKD and infection in ADPKD is a particularly serious complication. Here, we report a 79-year-old man with an intermittent high fever during 2 months and bulging mass at the right flank.