OBJECTIVES: Drugs for rare disorders rank among the most expensive medicines on a cost-per-patient basis. Economic issues to prioritize resource allocation decisions are increasingly being done by public health decision-makers in Brazil. Even though, there is not a clear threshold of willingness to pay. In this study, we did a narrative review of icatibant; an orphan drug considered the first choice for angioedema crisis and discussed the cost-effectiveness of its incorporation in Brazil. METHODS: The costs were estimated from the Brazilian Unified Health System perspective. Cost-effectiveness was evaluated using the incremental cost-effectiveness ratio (ICER). Considering the manufacturer`s prices, which represents the highest price for which a laboratory or drug distributor may sell a drug on Brazilian market, the average icatibant´s cost per patient crisis was estimated in 1,255.75 US dollars. The quality adjustment factors of each state in this study were derived from Brazilian health state preference (utility) data. We estimated the utilities according to two scenario assumptions: extreme pain and moderate anxiety (EuroQol 3L 11312; 0.644 utility) or extreme pain, moderate anxiety and being confined to bed (EuroQol 3L 31312; 0.26 utility). These two scenarios were compared to a complete resolution, a EuroQol 3L 11111 scenario; 1.0 utility).  RESULTS: Considering the longest time crisis being 51 hours, the total number of crisis hours per patient was estimated in a maximum 0.0021 years. We estimated an ICER between U$ 243,618.33 and U$ 506,397.66 per Qaly. CONCLUSIONS: In this study, an assumption of states that someone could expect during HA crisis was utilized for the first time. We considered icatibant not cost-effective. In Brazil, there is no well-defined threshold of cost-effectiveness for policymakers and payers. There is an urgent need to discuss and define the Brazilian society criteria and cost effectiveness limits related to orphan drugs.