We have achieved good results of renal transarterial embolization (TAE) therapy as an alternative to surgical nephrectomy to decrease renal size in autosomal polycystic kidney disease (ADPKD) patients with enlarged kidneys, but only in patients on dialysis. Renal transplantation is another treatment option, and patients who receive a donor kidney have achieved a reduction in kidney size after surgery. TAE has also been used in polycystic liver disease (PCLD), an extrarenal lesion of ADPKD. Although TAE is effective in some PCLD patients, others develop hepatomegaly, which leads to liver cyst infection and liver failure. Recently, liver transplantation has started to be performed in such patients. Initially, living donor liver transplants were performed from family members, but in 2010, deceased donor liver transplantation became available. This article gives an overview of the history of TAE for ADPKD and PCLD on the basis of our experience.