Objectives: Polycystic liver (PLD) is the most common extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). We have analyzed genetic and clinical factors related to the severity of PLD among the patients with Korean ADPKD. Methods: A total of 443 adult patients with typical ADPKD were enrolled from May 2019 to April 2021. Demographic, clinical, and laboratory data were collected at the initial study visit. Liver volumes were measured using Image J by one professional radiologist and adjusted for height (HtTLV) before analysis. The severity of PLD was defined by previous study: no cyst (Gr0), HtTLV<1000mL/m (Gr 1), HtTLV 1000-1800mL/m (Gr 2), HtTLV>1800mL/m (Gr 3). Targeted exome sequencing was done by gene panel including 89 ciliopathy-related genes. Genetic and clinical factors were compared between PLD groups. Results: The mean age was 45.7±13.4 years old and female comprised of 51.7%. The patients with liver cysts (n=131 in Gr 1, n=50 in Gr 2, n=34 in Gr 3) showed female predominance (59.1% vs. 44.7%, p=0.003) and higher frequency of ADPKD-related complication such as hypertension (87.4% vs. 71.5%, p<0.001), hematuria (20.0% vs. 12.7%, p=0.038), and proteinuria (27.9% vs. 14.9%, p=0.001) compared to those without liver cysts (n=228 in Gr 0). The patients in Gr 2 and 3 showed older age (48.8±9.6 vs. 45.3±12.8, p=0.023), higher number of pregnancy (2.2±1.5 vs. 1.7±1.5, p=0.064) and delivery of babies (1.7±0.9 vs. 1.3±1.1, p=0.039). Serum alkaline phosphatase was significantly higher in the patients with Gr 2~3 (76.6±56.0 vs. 57.1±26.6 IU/L, p=0.004). The proportion of PKD1 genotype or double variants were not different between PLD groups. Conclusions: PLD phenotype among the patients with ADPKD is less likely to be affected by genotype. Further genetic modifiers should be evaluated by whole exome sequencing or whole genome sequencing.