Autosomal dominant polycystic kidney disease (ADPKD) manifests with renal and extrarenal abnormalities and is inherited in an autosomal dominant fashion. In addition to multiple renal cysts, abnormalities such as liver cysts (80%), pancreatic cysts, splenic cysts, pulmonary cysts, berry aneurysms in the distribution of arterial circle of Willis (8%), colonic diverticula, mitral valve prolapse, etc., can be present. The condition will develop in half of the offspring of affected persons because of its 100% penetrance. Nephrolithiasis in patients with ADPKD is not infrequent and, given the importance of preservation of renal function in this subset of patients, a clear understanding of the management options available and their advantages and disadvantages is absolutely essential and critical in better patient outcomes. This article is an endeavor in this direction and provides a review of the current available literature.An electronic database search of Medline, Embase, and Cochrane library was performed to search for the available literature in January 2010 with no restrictions in terms of date or language. The search terms used were ADPKD, nephrolithiasis, percutaneous nephrolithotomy, shockwave lithotripsy, flexible ureterorenoscopy, congenital kidney disorders, etc., separately and in various combinations. The articles so extracted were scrutinized for relevance and selected for the review.