Introduction. Autosomal dominant polycystic kidney disease (ADPKD) is an inherited, multisystem disorder characterized by the progressive and diffuse development of multiple, bilateral renal cysts, frequently progressing to chronic kidney disease (CKD), and variable association with extrarenal abnormalities. ADPKD is a heterogeneous genetic disease caused by mutations in PKD1, PKD2, GANAB or DNAJB11 genes. Material and methods. The study included 53 patients with ADPKD in the Nephrology Department SCR, Chisinau in 2022. Clinical and paraclinical manifestations were documented; renal function was established according to KDIGO standards by assessing glomerular filtration rate (GFR) and albuminuria level. Pedigree transmission, penetrance, anticipation, parental imprinting in patients' families were assessed. Results. The study revealed the age of clinical manifestations onset- from 10 years to 65 years, duration of disease from 1 year to 50 years, clinical manifestations - 100% bilateral polycystic kidney disease, 73.5% arterial hypertension, 45% low back pain, 45% hepatic cysts, 26% urinary infections, 5.6% macrohematuria. 100% patients had CKD, 47.3% GFR <30ml/min/1.73 m2 and 28.5% albuminuria >300mg/g, 15% had end stage CKD. 58.5% patients inherited the disease from mother, 43% had other first-degree relatives with polycystic kidney disease. Earlier/more severe onset of clinical manifestations was demonstrated in 26% of cases in offspring vs parents, of which 70% inherited the disease from the mother. Conclusions. ADPKD is characterized by variable intra- and interfamilial phenotypic expression; male sex, anticipation, inheritance of the mutant gene from the mother, age and size of renal cysts, arterial hypertension, proteinuria and extrarenal manifestations are factors in disease progression.