PURPOSE: Percutaneous nephrolithotomy is standard therapy for upper tract calculi larger than 2 cm. However, the role of percutaneous nephrolithotomy in patients with autosomal dominant polycystic kidney disease has not been well evaluated. We report our experience with percutaneous nephrolithotomy in patients with autosomal dominant polycystic kidney disease. MATERIALS AND METHODS: We retrospectively reviewed the charts of all patients with autosomal dominant polycystic kidney disease and subsequent renal calculi managed by percutaneous nephrolithotomy from October 1981 to the present. RESULTS: We identified 9 patients. Percutaneous nephrolithotomy was performed in 11 kidneys. Flank pain was the presenting symptom in 6 patients. Average stone burden was 2.5 cm (range 1.6 to 3.6). Two access tracts were necessary in 5 kidneys. No intraoperative complications occurred. In 2 kidneys a second stage endoscopic procedure with ultrasonic lithotripsy was required to achieve stone-free status. Nephrostogram 24 hours after the final procedure showed no residual stone fragments in 9 of 11 kidneys (82%). The remaining 2 patients underwent percutaneous basket extraction to render them stone-free. There were no postoperative complications or recurrent stones. No patient required blood transfusion. Mean followup was 2.7 years (range 0.3 to 4). Mean calculated creatinine clearance was stable at 85.6 (range 45.9 to 126.6) and 89.5 mg/dl per minute (range 39.6 to 126.6) preoperatively and at last followup, respectively (p = 0.783). CONCLUSIONS: Autosomal dominant polycystic kidney disease increased operative complexity, the need for multiple percutaneous access tracts and the likelihood of repeat endoscopy. Despite the altered anatomy percutaneous nephrolithotomy was a safe, efficacious approach for autosomal dominant polycystic kidney disease. At last followup there was no stone recurrence and renal function was stable.