Abstract
Bilateral ADPKD is a well-known entity, but there are only a few reports on unilateral ADPKD in adults, most of which had associated contralateral agenesis. Further rare is the development of RCC in unilateral ADPKD. We present an exceedingly rare case of true unilateral ADPKD with normal contralateral kidney and an associated renal cell carcinoma in the same kidney.