Patients with autosomal dominant polycystic kidney disease (ADPKD) often suffer from abdominal symptoms. Although laparoscopic nephrectomy has been reported as a minimally invasive therapy, it is still technically demanding due to the large size of the kidneys. Transarterial embolization (TAE) is one of the alternatives, but there are only limited reports on its application in ADPKD. We describe a case in which bilateral nephrectomy was performed as a second-line treatment after TAE. One kidney was removed because a small feeding arterial branch was not completely embolized. The other kidney was removed due to infection. Retroperitoneoscopic nephrectomy was a good choice as a second-line modality in the case without infection because the volume of the kidney was reduced even with incomplete TAE, and adhesion after TAE was minimal. TAE is an effective choice in ADPKD patients without infection as a first-line treatment even when complete embolization is difficult, since nephrectomy after TAE is technically easier than removal of a fresh ADPKD kidney.