To evaluate the role of percutaneous nephrolithotomy in management of the patients with autosomal dominant polycystic kidney disease and associated nephrolithiasis.A total of 19 patients with autosomal dominant polycystic kidney disease and upper urinary tract stones were included in the study. One patient had bilateral renal stones. Most of the stones were located in the renal pelvis and/or calices with a stone size >2 cm in the largest diameter. All patients were treated by percutaneous nephrolithotomy at 2 centers. Therefore, 20 percutaneous nephrolithotomy procedures were performed. Most of the procedures were performed in 1 stage by the urology team under fluoroscopic guidance; 6 cases were done in 2 stages. The success rate and morbidity and mortality of the technique and hospital stay were recorded.The pelvicaliceal system was successfully approached in all cases but 1. The overall stone-free rate was 89.4%. Renal function improved in the patients who presented with azotemia and was stable in the others. No patient died. Complications occurred in 3 procedures and consisted of mild postoperative hematuria in 1, bleeding through the nephrostomy tube in 1, and low-grade fever in 1. The mean hospital stay was 3 days.Percutaneous nephrolithotomy is a safe and effective method for the treatment of patients with autosomal dominant polycystic kidney disease and associated upper urinary tract stones.