Polycystic kidney disease is a hereditary disorder of normal renal parenchyma differentiation due to numerous cysts in the kidney. There are two types of disease, namely autosomal dominant polycystic kidney disease (ADPKD), also known as the adult form, and autosomal recessive polycystic kidney disease (ARPKD), or infantile form of the disease. A major role in the pathogenesis of ADPKD is played by the mutation of the polycystic genes (PKD1 and PKD2); while the gene mutation for the fibrocystin/polyductin protein complex (PKHD1) is responsible for the onset of ARPKD. In the adult form of the disease, the symptoms usually occur around 30-40 years. The most common manifestations of ADPKD are arterial hypertension, low back pain, hematuria, palpable masses in the abdomen, kidney infection and nephrolithiasis. The disease often progresses to chronic renal failure and requires dialysis or kidney transplantation. The infantile form of the disease most commonly occurs in infants and includes oligohydramnion, massive renal enlargement, a "Potter" sequence with lung hypoplasia leading to respiratory failure, and congenital liver fibrosis. The main diagnostic method for polycystic kidney disease is ultrasound, which can clearly show cysts in the kidneys. Initial therapy consists of regulating blood pressure, increasing fluid intake and limiting sodium intake. Tolvaptan (inhibitor of V2 receptor) is common medication used in ADPKD, which promotes water excretion, increases vasoconstriction of afferent arterioles, and lowers eGFR. Treatment of infants with ARPKD is aimed at stabilizing respiratory function through mechanical ventilation, but some of them are requiring peritoneal dialysis in their first days. Therefore, early recognition and treatment of polycystic kidney disease plays a significant role in improving the patient's quality of life.