Findings demonstrate a frequent occurrence of abdominal wall hernias in polycystic liver disease with a predominance of umbilical hernias. Hepatomegaly represents an obvious disease-specific risk factor.

• This is a cross-sectional cohort study of 484 patients of which 40.1% (n=194) had an abdominal wall hernia.

• A clear predominance of umbilical hernias (25.8%, n=125) was evident while multiple hernias were detected to be present in 6.2% (n=30).

• In multivariate analysis, following were unveiled to be the risk factors: male sex (OR 2.727), abdominal surgery (OR 2.575) and disease severity according to the Gigot classification (Type 3 OR 2.853).

• In a subgroup of patients with known total liver volume, height-adjusted total liver volume was identified to be an independent polycystic liver disease specific risk factor (OR 1.363).

• Older people (62.1 vs 55.1) and more commonly males (22.0 vs 50.0%) had multiple hernias.