Historically, cystic fibrosis has been a devastating disease, with patients rarely living beyond childhood. Recent advances during the last two decades, however, have led to an improved understanding of the disease on a molecular level. Such understanding has in turn led to new treatments, longer life expectancy, as well as new challenges in the management of both pulmonary and extrapulmonary manifestations of cystic fibrosis. The otolaryngologist caring for patients with cystic fibrosis is thus challenged with the medical and surgical management of chronic sinusitis and sinonasal polyposis in these challenging circumstances.