ADPKD is the most common cause of intrarenal cystic degeneration and is the cause of end-stage renal disease in 10% of dialysis patients.ADPKD is a genetic abnormality associated with mutations on chromosomes 16 and 4. Autosomal dominant inheritance results in a 50% chance of having a child with ADPKD, but there is a wide variation in disease prevalence and age of onset. Roughly 25% of patients have no apparent family history, either because they (they) have a spontaneous mutation in their chromosome or because their (their) affected father or mother died before the disease developed. The kidneys of ADPKD patients have normal function and structure until the age of 10 years. However, anatomical disorders gradually appear between the ages of 10 and 20, and patients may not become symptomatic until the age of 30. Typically, patients begin to have symptoms of pain, hematuria or urinary tract infection due to the increase in the number and size of cysts between 30 and 40 years of age, when most patients have near normal renal function; they do not develop elevated BUN and creatinine until 40 to 50 years of age, but before that the kidney’s ability to concentrate urine has decreased. Elevated creatinine is a late manifestation of ADPKD, when the kidney disease deteriorates and approximately 50% of patients have developed end-stage renal disease (ESRD) by age 60. ADPKD is often accompanied by multiple abnormalities, with congenital cystic degeneration being more common. Approximately 50% of patients have a combination of liver cysts; they have normal liver function but may experience discomfort due to the large cysts. The early manifestations of liver involvement are often anorexia, nausea and food reflux. There may also be cystic lesions in the pancreas, ovaries, spleen and subarachnoid space, while the most dangerous comorbidity is intracranial aneurysms, which may have asymptomatic intracranial lesions in about 40% of patients and rupture of intracranial lesions at an earlier age in patients with ADPKD than in the general population. It appears that there is a group of patients who are prone to have both ADPKD and aneurysms, and therefore patients with ADPKD who have a family history of aneurysm or acute cerebrovascular accident should be screened and treated surgically once an intracranial lesion is detected. Recently, ADPKD has also been found to be associated with heart valve disease. The parenchyma of the affected kidney is replaced by hundreds of cysts ranging in size from a few millimeters in diameter to several centimeters in size, surrounded by cuboidal or columnar epithelial cells, which can involve any part of the full length of the renal unit. These cysts originate from the renal tubules and as they grow they gradually lose their tubular structure and become independent cyst-like structures. The symptoms of ADPKD are complex and often difficult to evaluate. Pain is most common and often precedes the palpable enlargement of the kidney. The nature of the pain is important because the mass action of the enlarged kidney often causes a dull pain, which may be caused by pulling on the kidney’s peritoneum or compression of surrounding organs. Sharp pain, on the other hand, implies rupture of the cyst, infection, bleeding, obstruction due to cyst compression, stones or clots can cause colic. Hematuria is also common and suggests cyst rupture or other possibilities such as stones, infections and tumors. Because a definitive diagnosis is often not obtained on imaging, which is mainly due to significant deformation of the kidney, ADPKD should be considered in patients presenting with the above symptoms. enhanced CT scan is very useful for the differential diagnosis of possible infection, bleeding and stones. Ultrasound is also useful for diagnosis, but large cysts and calcification of the cyst wall interfere with the differential diagnosis of renal stones and hydronephrosis by ultrasound. IVP is limited due to renal insufficiency in many patients, but it will help to determine the site of ureteral obstruction. However, CT is still the most important because it is a good differentiator between cysts and dilated calyces, and it can localize infected foci more accurately than other imaging. More than half of the patients with ADPKD have urinary tract infections, mainly in women, and in fact, urine bacteriological cultures may be negative, depending on the site of infection. Both purulent cysts and obstructed infected calyces do not communicate with the urinary tract, and clean midstream urine cultures cannot detect infectious organisms. Therefore, further CT examination is necessary for the determination of cases with negative urine culture and the need to exclude infection, and the treatment of infection is associated with morbidity and mortality in ADPKD, requiring the application of lipophilic antibiotics and/or thorough drainage therapy, and should not be routinely performed for urinary tract instrumentation, which may cause endogenous infection.