Polycystic liver disease presenting as pruritus

Polycystic liver disease (PCLD) is a rare autosomal dominant disorder which usually occurs in association with autosomal dominant polycystic kidney disease. Biliary obstruction is a rare complication of PCLD. Treatment options include percutaneous aspiration and sclerosis, surgical deroofing of cysts, liver resection or liver transplantation. Medical treatment involves the use of somatostatin analogues or mTOR inhibitors. We present a case of PCLD presenting for the first time with longstanding pruritus as the only symptom.