Introduction: autosomal polycystic kidney disease (ADPKD) is a hereditary orphan disease, with an average world prevalence of 2.7 per 100,000 inhabitants. Until recently, treatment was limited to the control of clinical manifestations, but since 2015 with the appearance of tolvaptan, the disease has been managed and the introduction of venglustat and lanreotide acetate as treatment makes it necessary to review therapeutic options. Methods: narrative review in PubMed, Cochrane Library, OVID, EBSCO and Google Scholar. Results: Were obtained 92 articles in the search strategy, of which five were included in the qualitative analysis. The TEMPO 3: 4 Study showed that tolvaptan reduced the annual growth rate in the total kidney volume from 5.5% to 2.8% (p <0.001), reducing the deterioration of the filtration rate. Glomerular tissue estimated at −3.70 to −2.72 mL/min/1.73 m2 (p <0.001) compared to placebo. THE DIPAK 1: evidence that lanreotide reduces kidney volume compared to placebo (4.15% vs. 5.56%; per year; 95% CI: -2.41% to -0.24%; p= 0.02). Venglustat clinical trials showed preliminary improvement in the annualized rate of change in total kidney volume (TKV) and estimated glomerular filtration rate (eGFR), according to the results of preclinical studies, however, they are still in study. Conclusions: ADPKD is a pathology with few therapeutic options and with recent treatments incorporated, currently under investigation. This article sets the guide for future study design quantitative.