OBJECTIVES: The increase in premium-priced orphan drugs coupled with health care budget constraints will pressure managed care plans to consider restricting market access. Coverage and reimbursement of ten FDA-designated orphan drugs (alpha-glucosidase, bexarotene, bosentan, galsulfase, idursulfase, iloprost, laronidase, mecasermin, nitisinone, plerixafor) were analyzed for seven popular Medicare PDP (BCBS Rx, AARP Medicare Rx Preferred, Cigna Medicare Rx Plan One, Humana Enhanced, Aetna/CVS, EmblemHealth, UnitedHealthcare). METHODS: Formulary tier structure, monthly retail costs and utilization restrictions (UR)—pre-authorization (PA), quantity limits (QL) and step therapy (ST)—were obtained from CMS (www.medicare.gov).  UR were assigned point values reflecting most to least restrictive—QL, 1; ST, 2; PA, 3; exclusion from formulary, 6.  Each drug-plan combination can be assigned a maximum of 6 points.  Disease prevalences were obtained from a variety of sources. RESULTS: Monthly retail prices ranged from $782.45 (laronidase) to $11,845.25 (mecasermin). Iloprost was most frequently excluded from formularies (2) and was subject to PA from the remaining five plans.  The most frequently PA drug was mecasermin (6).  There was slight negative correlation between price and prevalence of the disease for which the drugs were indicated (r2= 0.080).  There was a slight positive correlation between price and the number of UR points (r2= 0.046).  There was virtually no correlation between UR points and disease prevalence (r2= 0.004).  However, there is a moderate correlation between the number of plans that implement PA for a drug and that drug’s price (r2= 0.322). CONCLUSIONS: With a wide range of drug prices, there is virtually no correlation between the number of UR points, drug price and disease prevalence.  However, it is clear that PA is perceived to be the most effective cost-saving measure as it is used most for the more expensive drugs.