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Introduction: Severe asthma is characterized by frequent exacerbations, symptoms limiting daily activities and nocturnal symptoms. It requires the continuous use of medications, at high doses, and, sometimes, continuous use of oral corticosteroids, representing a significant burden to health system and society. This systematic review sought to address economic data related to severe asthma in Brazil., Method: In June 2014, electronic searches were conducted to identify relevant publications. Quality criteria were developed and applied to each selected study. In order to compare results across the selected studies, costs were refined to an annual basis, grouped according to the study perspective, inflated and converted to 2014 USD., Results: Cost analyses from the Brazilian public health system perspective were derived from two studies and showed an average annual hospital cost per patient of 135 USD and 733 USD, respectively. From the family perspective, average annual direct costs per patient varied from 764 USD to 929 USD., Conclusion: Hospitalizations and medications seem to be the most important resources funded by the Brazilian public health system and by patients and their families. Although further studies are necessary, as information on cost of this disease is scarce in Brazil, these findings suggest that there is a potential room for improving severe asthma care among Brazilian patients.