Polycystic liver disease (PLD) comprises a spectrum of inherited disorders characterised by progressive cyst development and highly variable clinical manifestations. A significant subset of patients develops debilitating symptoms and despite advances, a unified treatment algorithm is lacking.

To review current evidence for medical, interventional and surgical management of PLD and identify gaps preventing an integrated, evidence-based care pathway.

Somatostatin analogues (SSAs) are the principal disease-modifying therapy, producing modest but reproducible liver-volume reductions of 3–7% and clinically meaningful symptom improvement in selected patients with diffuse small-to-medium cystic disease. Other pharmacologic strategies have shown limited or inconsistent benefit and currently have no established role outside research settings. Interventional radiologic and surgical options provide more substantial debulking but are phenotype-dependent. Partial hepatectomy offers the largest volume reduction but carries high morbidity and liver transplantation (LT) remains the only curative option for advanced disease, with excellent long-term outcomes but significant perioperative risk. Across all modalities, heterogeneous endpoints limit meaningful comparison and hinder integration of therapies into a unified treatment pathway.

A phenotype-driven management framework and coordinated research strategy incorporating standardised volumetrics, symptom scoring and prospective multicentre cohorts are urgently needed to define optimal sequencing of medical, interventional and surgical therapies in PLD.