Autosomal-dominant polycystic kidney disease (ADPKD) is the most common inherited renal cystic disease. It is characterised by the development of renal parenchymal cysts and a variety of other extrarenal manifestations. Pelvi-ureteric junction (PUJ) obstruction has not been described in association with ADPKD in the literature. We present a case of a 23-year-old man presenting with bilateral flank pain. On evaluation he was diagnosed to have ADPKD with bilateral renal calculi and left-sided PUJ obstruction. He underwent successful right percutaneous nephrolithotomy and left laparoscopic dismembered pyeloplasty with simultaneous stone removal.