Polycystic liver disease (PLD) is usually associated with polycystic kidney disease but may also occur as an isolated finding in a rarer genetically distinct disease. In either case, the cyst burden will progress over time and, in rare cases, may affect liver function or become symptomatic due to massive hepatomegaly. The character, distribution, location, and size of hepatic cysts are important. Computed tomography, magnetic resonance imaging, or ultrasonography may provide the surgeon with valuable preoperative information, such as the location of infected or hemorrhagic cysts that may be responsible for symptoms. Less invasive cyst aspiration or fenestration may provide temporary relief from dominant or symptomatic cysts, but these cysts will recur in up to 75% of patients. Cyst fenestration with partial hepatic resection and liver transplantation are two therapies that provide more permanent resolution of symptoms in patients with extensive hepatic involvement. However, the higher risk of complications associated with more aggressive surgical therapy must be considered when determining the appropriate therapy for a given patient. Knowledge of the cyst patterns and available treatment options in patients with PLD will help the radiologist provide the referring clinician with important information for therapeutic decision making. ©RSNA, 2006.