This article updates current knowledge on epidemiology, risk factors, triggers, and management of anaphylaxis in patients with mastocytosis. Hyperactive mast cells and higher number of effector mast cells are speculated to facilitate anaphylaxis in this condition. In children, increased risk is limited to those with extensive skin involvement and high tryptase. In adults, manifestations of anaphylaxis are severe with high frequency of cardiovascular symptoms. Hymenoptera stings are the most common triggers for these reactions; however, idiopathic anaphylaxis and reactions to food or drugs occur. Patients with mastocytosis should be informed about risk of anaphylaxis and prescribing emergency self-medication and installing emergency preparedness before general anesthesia is considered.