The purpose of this study was to evaluate the efficacy of laparoscopy in managing patients with abdominal symptoms from autosomal dominant polycystic kidney disease (ADPKD). From April 1993 to July 1995, four patients with ADPKD underwent seven laparoscopic procedures: five cyst decortications were performed in two patients using a laparoscopic ultrasound unit and two laparoscopic nephrectomies were performed in two patients with end-stage renal failure. The mean operative time was 207 minutes for laparoscopic cyst decortication and 272 minutes for laparoscopic nephrectomy. The two nephrectomy specimens were 2,200 g and 1,750 g, respectively. The mean intraoperative blood loss was 85 mL. The patients resumed their oral intake within 10 hours after laparoscopic cyst decortication and within 16 hours after laparoscopic nephrectomy. The mean amount of parenteral analgesics required postoperatively was 12 mg morphine sulfate for cyst decortication and 30 mg morphine sulfate for nephrectomy. The mean hospital stay was 3 days for cyst decortication and 3.5 days for nephrectomy. The patients returned to their usual activities after an average of 2 weeks. Based on pain analog scales, all the patients have shown marked reduction in their symptoms (average, 90%) during an average follow-up period of 6.6 months. Laparoscopic cyst decortication and nephrectomy are effective minimally invasive treatment options for patients with adult polycystic kidney disease who are experiencing abdominal symptoms due to marked renal enlargement. We believe that by using a laparoscopic ultrasound unit, most renal cysts may be safely removed, and if need be, even "giant" kidneys can be removed laparoscopically. To the best of our knowledge, the two nephrectomy specimens in this study represent the largest kidneys removed laparoscopically to date and the first laparoscopic nephrectomies in ADPKD patients.