Isolated polycystic liver disease (IPLD) is a rare genetic condition characterized by the presence of multiple liver cysts with no association with polycystic kidney disease. Most patients are asymptomatic and acute complications (cyst torsion, bleeding, infection) are uncommon.Imaging techniques, including abdominal ultrasounds, computerized axial tomography, and magnetic resonance imaging, represent a vital diagnostic modality. They are also useful for therapy support in this disease. Below we report a peculiar case of a female patient recentlydiagnosed with IPLD who, having received treatment with ultrasoundguided percutaneous drainage and sclerotherapy for a giant liver cyst, showed symptom and laboratory improvement.