We performed a retrospective study of seven patients with polycystic liver disease who underwent stenting of the inferior vena cava for intractable ascites. All patients had symptomatic ascites and inferior vena cava stenosis demonstrable by venography. The mean pressure gradient across the inferior vena cava stenosis before stenting was 14.5 mm Hg (range 6-25 mm Hg) and significantly decreased to a mean pressure gradient of 2.8 mm Hg (range 0-6 mm Hg, p = 0.008) after stenting. Two patients also had stenting of hepatic venous stenoses after unsuccessful inferior vena cava stenting. After a mean follow-up of 12.2 months (range 0.5-39.1 months), five of the seven patients have had maintained clinical improvement, defined as decreased symptoms, diuretic requirements, and frequency of paracentesis. Four patients have required no further intervention. The other patient was lost in follow-up. Patients with clinical improvement had an overall larger mean pressure gradient before stenting (19.2 vs. 9.8 mm Hg) and a larger Delta pressure gradient (15.8 vs. 7.8 mm Hg) compared to those in whom stenting was unsuccessful. These results suggest inferior vena cava stenting is safe and effective and should be considered as a first-line intervention in the treatment of medically intractable ascites in select patients with polycystic liver disease.