Kounis syndrome is a ubiquitous condition, representing a natural paradigm between coronary artery vasospasm, plaque rupture and anaphylactoid reactions. Kounis syndrome is underdiagnosed, as the inflammatory cells releases, in both allergic and non-allergic acute coronary syndrome, are the same. The syndrome is induced by various causes such as drugs, foods, environmental exposure, stents. There are three variants of Kounis syndrome: vasospastic allergic angina, allergic myocardial infarction, and stent thrombosis. All patients with systemic allergic manifestations correlated with clinical, laboratory and electrocardiography findings of acute myocardial infarction must be investigated for Kounis syndrome. The therapeutic management of Kounis syndrome represents a challenge, as it is necessary to treat both the cardiac and allergic events. After the acute event, a complete cardiological follow-up is mandatory, skin tests, verification of allergies to food, insect stings, drugs and environment agents. The awareness of etiology, epidemiology, pathogenesis, signs and symptoms is important for its diagnosis, treatment, prognosis and prevention.