We determined whether telmisartan ameliorates the three major symptoms of ARPKD, polycystic kidney disease, fibrocystic liver disease, and hypertension by its PPAR-γ agonist and angiotensin II type 1 receptor blockade activity in this orthologous rat model of human ARPKD. The gene discussed is PPARG; the disease is autosomal recessive polycystic kidney disease.