IntroductionWe focused on neutrophil gelatinase‐associated lipocalin (NGAL) and autosomal dominant polycystic kidney disease (ADPKD) progression.MethodsADPKD patients with an estimated glomerular filtration rate (eGFR) ≥ 30 mL/min/1.73 m2 were included. Serum NGAL level and NGAL to eGFR ratio (NGR), height‐adjusted total kidney volume (hTKV) were assessed initially. Patients were followed‐up for 5 years.ResultsSixty one patients were enrolled and initial eGFR was 73.6 (48.9–101.5) ml/min/1.73m2. EGFR declined by 3.7 mL/min/1.73m2 per year. Thirty four patients (55.7%) exhibited rapid progression. Rapid progression group had lower serum NGAL levels (p < 0.001) and higher hTKV (p < 0.001). Lower serum NGAL level was a risk factor for rapid progression (p < 0.001). NGR was not associated with rapid progression. Serum NGAL level was predictive in for rapid progression ROC analysis (cut‐off <10.62 ng/mL).ConclusionRelatively lower serum NGAL levels can predict worse outcomes in ADPKD and can provide risk stratification in patients with ADPKD.