Angiotensin-converting enzyme (ACE) inhibitors frequently are prescribed for the management of cardiovascular disorders. In addition to their therapeutic potential, ACE inhibitors are associated with numerous adverse effects, some of which may be lethal. Angioedema is an uncommon but serious acute event that may affect any individual taking an ACE inhibitor. This review outlines the advances in our understanding of the pathogenesis, clinical presentation, and management of this medical emergency.