Autosomal dominant polycystic kidney disease is the most common inherited kidney disease whose main feature is cystic degeneration on both kidneys, which eventually leads to it's enlargement and failure. The PKD1 and PKD2 genes play a decisive role in the pathogenesis of autosomal dominant polycystic kidney disease. In 85% of cases it is a mutation of the PKD1 gene, while in the remaining 15% it is a mutation of the PKD2 gene. The diagnosis is made on the basis of a positive family history, age and number of cysts in the individual. Fortunately, an early diagnosis allows for proper control of kidney function and timely treatment of complications, and can prolong the onset of kidney failure. Renal manifestations may include kidney stones, various urinary tract infections as well as hematuria, while extrarenal onesare manifested through pain, hypertension, liver cysts, diverticulosis, and left ventricular hypertrophy. Hypertension as the most common and earliest manifestation of the disease occurs in most patients who did not have any accompanying symptoms related to renal function. Treatment of autosomal dominant polycystic kidney disease includes correction of diet and fluid intake, treatment of pain, hypertension, and concomitant complications. The key role of the nurse in providing nursing care is a holistic approach and education of the patient and/or his family about the planned and implemented procedures through treatment, thereby improving the quality of life of the chronically ill patient.