[2] The much rarer autosomal-dominant polycystic liver disease will progress without any kidney involvement.

[3] Polycystic liver disease comes in two forms: autosomal dominant polycystic kidney disease (with kidney cysts) and autosomal dominant polycystic liver disease (liver cysts only).

[citation needed] Most patients with PLD are asymptomatic with simple cysts found following routine investigations.

After confirming the presence of cysts in the liver, laboratory tests may be ordered to check for liver function including bilirubin, alkaline phosphatase, alanine aminotransferase, and prothrombin time.

[2] Patients with PLD often have an enlarged liver which will compress adjacent organs, leading to nausea, respiratory issues, and limited physical ability.