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Purpose Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, and 20% of patients eventually require nephrectomies due to compressive symptoms or renal-related complications. Traditionally, nephrectomies were performed via the open approach in view of space constraints. We evaluate our institution's outcomes for laparoscopic nephrectomy (LN) for ADPKD. Materials and methods We retrospectively reviewed 33 patients with ADPKD who underwent nephrectomies from November 2005 to December 2016 at a tertiary institution. Preoperative kidney volume was calculated via the ellipsoid method by using computed tomography scan. Results The median age was 51.0 years (interquartile range [IQR], 44.5-56.0 years). Sixteen patients (48.5%) underwent open nephrectomy (ON), 15 patients (45.5%) had LNs, and 2 patients (6.1%) had laparoscopic converted to ON due to dense adhesions. Thirteen patients had bilateral while 18 patients had unilateral nephrectomies. Median kidney volume in the open group was 1,042 cm3 (IQR, 753-2,365 cm3) versus 899 cm3 (IQR, 482-1,914 cm3) in the laparoscopy group and did not differ significantly. The operative time was comparable between both groups. Patients who underwent LN had lesser blood loss (350 mL vs. 650 mL; 95% confidence interval [CI], 1.822-3.533; p=0.016) and shorter length of hospital stay (4.0 days vs. 6.5 days; 95% CI, 1.445-5.755; p=0.001) compared to patients who underwent ON. Both groups had similar low morbidity rate and no mortality. Conclusions LN for ADPKD is a safe and effective alternative to ON independent of kidney size with comparable outcomes and benefits of minimally invasive surgery.