ABSTRACT Elexacaftor/tezacaftor/ivacaftor is a novel “triple therapy” drug which has been shown in clinical trials to reduce morbidity and mortality in cystic fibrosis patients. Few reports of serious cutaneous adverse reactions to elexacaftor/tezacaftor/ivacaftor leading to drug discontinuation have been previously documented, all occurring within 1 month of drug initiation. Herein we present a novel case of delayed bullous drug eruption, arising 2 years after elexacaftor/tezacaftor/ivacaftor initiation, with subsequent successful drug re‐introduction. Documentation of the possible serious cutaneous adverse effects of elexacaftor/tezacaftor/ivacaftor will importantly aid understanding of the potential for drug re‐challenge in these cases.