Abstract: Objective To observe and evaluate the progression of autosomal dominant polycystic kidney disease (ADPKD) by measuring the total kidney volume (TKV) with magnetic resonance imaging (MRI). Methods A total of 166 ADPKD patients without end-stage renal disease were enrolled in this study. The data on history, family history, physical examination, routine blood and urine examinations, biochemical examination and abdominal MRI were obtained. The TKV was accurately measured after three-dimensional reconstruction. The correlation between TKV and various parameters was analyzed, and the reason of rapid increase of renal volume was analyzed through the annual growth rate of TKV. Results The TKV of female patients was significantly smaller than that of male patients (P<0.05), and the annual growth rate of TKV was slightly lower than that of male patients (P>0.05). TKV in ADPKD patients was increased and positively correlated with age (P<0.01), but negatively with eGFR (P<0.01). There was no significant difference in age, sex and body mass index between slow growth TKV (TKV annual growth rate<5%) and fast growth TKV (TKV annual growth rate>5%) (P>0.05); The baseline TKV of the fast growth was significantly larger than that of the slow growth (P<0.01). Conclusions Accurate TKV measurements provide a measure of ADPKD progression and can be used to assess disease progression and the efficacy of potential treatments for ADPKD.