Chronic rhinosinusitis with nasal polyposis (CRSwNP) represents a subset of chronic sinusitis with various causes. Some forms of the disease are driven by allergy, often in association with asthma. Refractory CRSwNP can be associated with cystic fibrosis and other clinical syndromes. More recent literature is presented regarding roles of innate immunity and superantigens. Effective treatment of CRSwNP requires careful endoscopic sinus surgery followed by an individualized treatment plan that often includes oral and topical steroids. Recidivism of polyps is common, and patients require long-term follow-up.