Summary

Polycystic liver disease is the most common extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). Hepatic cysts are typically incidental findings, with occasional complications including cyst haemorrhage, infection and rupture. In contrast to the typically benign course of polycystic liver disease, we present a rare case of fatal decompensated liver failure in a patient with ADPKD. This is a case of a 58-year-old man with end-stage renal disease on haemodialysis presenting with new-onset ascites and decompensated liver failure following bilateral nephrectomy. Cirrhosis in ADPKD is a late manifestation of the disease, but it should be considered in the perioperative risk of patients with ADPKD.