Drug-drug interactions inducing adverse drug reactions remain a significant, avoidable health risk to cystic fibrosis (CF) patients. With increasingly complex CF exacerbation treatment regimens being used, the risk of clinically important drug-drug interactions has in turn increased. Cardiovascular medications have reportedly been commonly implicated in severe CF exacerbation drug-drug related reactions. Therefore, knowledge of these potential drug-drug interactions is essential for improvement of medication safety and preventing patient harm. Drug-drug interactions can be classified into either pharmacokinetic or pharmacodynamic. This article, using the British National Formulary, details the drug-drug interactions between commonly prescribed CF exacerbation medications and cardiac medications. For each respective drug-drug interaction, the potential consequence, management strategy and interaction severity have been described.