Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of chronic kidney disease, causing a high rate of morbidity and mortality worldwide. Advances in molecular genetics and imaging techniques have improved diagnostic and prognostic tools. Tolvaptan is a disease-modifying treatment with a strong evidence base, so its early use is recommended in patients at high risk of renal failure. Treatment with tolvaptan slows the growth of renal cysts, reduces the decline in glomerular filtration rate (eGFR) by 26% and kidney growth by 45% in early-stage patients. They have been studied in randomized clinical trials with thousands of patients, where they have also been shown to reduce kidney pain and urinary tract infections. Screening and management of complications such as cystic kidney infections, intracranial aneurysms, and polycystic liver disease as part of comprehensive care is important.