ADPKD is a systemic disorder, associated with numerous extrarenal manifestations, including polycystic liver disease (PCLD) and other gastrointestinal manifestations, as well as pancreatic cysts, diverticular disease, inguinal and ventral hernias which play a significant role in disease burden, particularly in the advanced stage of ADPKD. In most cases the natural history of ADPKD goes through a long period of stability followed by a progressive decline in renal function. The coexistence of hypertension, cyst infections and nephrolithiasis can influence and accelerate the progression of kidney failure. The early diagnosis and prevention of these conditions are of foundamental importance. Nephrologists should know how to recognize and handle other clinical manifestations related to ADPKD like haematuria, renal cell carcinoma and intracranial aneurysms (ICA).