Adult polycystic kidney disease (APKD) is a common genetic disease and one of the important reasons of end stage renal failure. Although renal multiple cysts are clearly an important manifestation of APKD; other systemic manifestations are both common and clinically important. The authors reviewed 205 cases from 180 APKD families (107 male 98 female). Their age ranged from 10 to 71 years. Renal cyst is one of the many renal manifestations. Hypertension, hematuria and flank pain are its major complications. Hepatic cysts, pancreatic cysts, cardiac valvular lesions, intracranial aneurysms and splenic cysts are included in the array of systemic manifestations.