Despite significant progress in therapy for cystic fibrosis (CF), most patients with the disease still die before the age of 30 years. Published discussions and descriptions of end-of-life care of patients with CF have been few, but interest in these issues is increasing. Descriptions of end-of-life care for persons with CF have begun to appear, and conflicting models of care have been proposed. Advanced care planning offers an opportunity for persons with CF to actively influence the type of care they will receive. Still, more empiric research and ethical discussion is needed to facilitate optimal end-of-life care of patients with CF.