Autosomal dominant polycystic kidney disease (ADPKD), characterized by the development of multiple cysts in the kidneys and other organs, is the most common hereditary renal disorder and the fourth leading cause of end-stage renal disease. In adults with a positive family history, the diagnosis of ADPKD is made based on the radiologic evidence of bilateral, fluid-filled renal cysts. Furthermore, initial symptoms including pain, increased thirst, polyuria, nocturia, and increased urinary frequency may lead to the diagnosis of ADPKD. An easily accessible, applicable, and cost-effective biomarker is needed to predict the clinical course of ADPKD due to its progressive pattern. Urine is an easily obtainable and widely used test specimen for diagnosis and follow-up in several renal diseases. Thus, the aim of the present study was to review and assess new urinary biomarkers and urinary findings in ADPKD.