Aspirin-exacerbated respiratory disease (AERD) refers to the combination of asthma, chronic rhinosinusitis with nasal polyposis, and acute upper and lower respiratory tract reactions to the ingestion of aspirin (acetylsalicylic acid, ASA) and other cyclooxygenase-1 inhibiting non-steroidal anti-inflammatory drugs. AERD affects 0.3-0.9 % of the general population. AERD generally occurs due to abnormalities in mediators and expression of arachidonic acid biosynthesis. Local IgE responses to staphylococcal enterotoxins may also be responsible for eosinophilic activation in the nasal polyp tissues of AERD patients. Clinical features of AERD include the onset of nasal congestion with anosmia, progressing to chronic pansinusitis and nasal polyps that regrow rapidly after surgery. Aspirin desensitization, Leukotriene-modifying agents, biologic agents, management of asthma, chronic rhinosinusitis, and nasal polyposis are recommended as treatment modalities. Immunotherapy is prescribed only to those AERD patients who experience clear seasonal or perennial allergy symptoms in addition to the symptoms attributable to chronic nasal polyposis. There are also investigational and dietary therapies. In this review, the important aspects of AERD will be presented, along with a literature survey.