Refractory ascites is uncommon in autosomal dominant polycystic kidney disease (ADPKD) but it usually makes the patient physically and psychologically handicapped. Two uremic ADPKD patients in our hospital developed refractory ascites after 1 year on hemodialysis. The refractory ascites was due to portal hypertension, which was caused primarily by portal outflow obstruction due to the numerous enlarged cysts in the liver and secondarily by increased portal inflow. We attempted continuous ambulatory peritoneal dialysis (CAPD) to treat the 2 patients and obtained satisfactory results. Not only was the refractory ascites well controlled, but also the portal hypertension disappeared. Based on our experience, we think CAPD could serve as a very effective therapy to treat the refractory ascites of portal hypertension due to polycystic liver in uremic ADPKD patients.