OBJECTIVES: ADPKD is a rare monogenic renal disorder impacting approximately 1:2500 individuals among the general US population. Cardiovascular Disease is the most common cause of mortality among ADPKD patients. Hypertension has been documented as a predictor of disease progression and severity. Few intervention studies have examined the role of antihypertensive therapies in ADPKD and therefore a need to synthesize the literature exists. The objective is to determine if novel Angiotensin II Receptor Blockers(ARBs) are more cost-effective than Angiotensin-Converting Enzymes(ACE) Inhibitors as first line treatment in ADPKD. METHODS: A Markov-state decision model was constructed for estimation of cost and outcome benefits in hypertensive ADPKD patients. Transition probabilities were extrapolated from a retrospective cohort study comparing Chronic Kidney Disease(CKD) stage transitions in ADPKD patients. Annual pharmaceutical costs pending average daily dose per CKD stage was extracted from a large US healthcare claims database. Median total health care costs per CKD stage or transplant were extracted from published data. Time horizon was set to 30 years with 1-year duration to cycle shift. Cost-effectiveness analysis was conducted to estimate the incremental cost-effectiveness ratio(ICER) of ACE-I vs. ARB per additional year of prevented transplant and/or death. Sensitivity analysis was conducted using a one-way probabilistic analysis with 10% variation in transition/mortality probabilities and cost. RESULTS:  Total annual health care costs accrued after 30 years among ADPKD patients taking ACE-I was estimated to be approximately $3,505,028.41 compared to ARB at $3,644,327.65. Life expectancy was increased by 1.39 years among patients taking ACE-I. Approximate 10-year survival in patients taking ACE-I was 47% compared to ARB at 34%. CONCLUSIONS: ACE-I dominated ARB and displayed greater cost-effectiveness due to lower cost and increased capacity to prolong years of life without transplant or death among hypertensive ADPKD patients. This model validates the use of ACE-I as first line treatment for hypertension management in ADPKD patients.