The CFTR modulators currently available in clinic for CF are: ivacaftor, lumacaftor/ivacaftor, tezacaftor/ivacaftor, elexacaftor/tezacaftor/ivacaftor (Vertex Pharmaceuticals, MA, USA) and are currently revolutionizing the management of patients with CF, particularly those with at least one F508del variant (up to 85% of patients worldwide). The gene discussed is CFTR; the disease is cystic fibrosis.