Eosinophilic granulomatosis with polyangiitis (EGPA) characterized by the coexistence of asthma, eosinophilia and characteristic histopathologic changes, is one of the primary systemic small vessel inflammation of unknown etiology. Cardiac involvement in the course of EGPA occur in 17–92% of patients affecting poor prognosis and acting major cause of mortality in this group of patients. EGPA should always be considered in the differential diagnosis of acute heart failure, especially in young patients with no risk factors for coronary heart disease, and in patients with the presence of asthma, allergic rhinitis or nasal polyps. A rare complication of cardiac involvement is the thrombus formation in the left ventricular apex requiring anticoagulation. We report a case of 49 year old man with cardiac involvement that mimic acute coronary syndrome and led to the diagnosis of EGPA.