The May-Hegglin anomaly (MHA) is a rare autosomal dominant platelet disorder characterized by thrombocytopenia, giant platelets and leukocyte inclusion bodies. Many patients affected by the MHA have a marked hemorrhagic tendency, a well known contraindication to thrombolytic and anticoagulant therapies. We report a case of a 56-year-old woman with the MHA, referred to our department with an evolving acute ST-elevation myocardial infarction. The patient underwent urgent coronary angiography revealing the acute occlusion of the distal left anterior descending coronary artery, treated with a thrombus aspiration system. In view of the absence of residual stenosis, no balloon dilation and stent deployment were performed. No antiaggregant and anticoagulant therapy was administered. The procedure has been successful, the hospital course was uneventful and the patient was discharged 5 days later. At a 30-day follow-up the patient was asymptomatic and in a good hemodynamic state. To the best of our knowledge, this report is the first description of managing a myocardial infarction in a patient affected by the MHA in the reperfusion era.